Matheson died just days before he was due to receive the Visionary award at the 39th Saturn Awards ceremony. As a tribute, the ceremony was dedicated to him and the award was presented posthumously. Academy President Robert Holguin said "Richard's accomplishments will live on forever in the imaginations of everyone who read or saw his inspired and inimitable work."

According to film critic Roger Ebert, Matheson's scientific approach to the supernatural in I Am Legend and other novels from the 1950s and early 1960s "anticipated pseudorealistic fantasy novels like Rosemary's Baby and The Exorcist."

In 1952, Matheson married Ruth Ann Woodson, whom he met in California. They had four children. Bettina Mayberry, Richard Christian Matheson, Chris Matheson and Ali Matheson.

Matheson's first novel to be published, Someone Is Bleeding, appeared from Lion Books in 1953. In 1960, Matheson published The Beardless Warriors, a non-fantastic, autobiographical novel about teenage American Soldiers in World War II. It was filmed in 1967 as The Young Warriors though most of Matheson's plot was jettisoned. During the 1950s he published a handful of Western stories (later collected in By the Gun); and during the 1990s he published Western novels such as Journal of the Gun Years, The Gunfight, The Memoirs of Wild Bill Hickok, and Shadow on the Sun.

His other early novels include The Shrinking Man (1956, filmed in 1957 as The Incredible Shrinking Man, again from Matheson's own screenplay) and a science fiction vampire novel, I Am Legend (1954) (filmed as The Last Man on Earth in 1964, The Omega Man in 1971 and I Am Legend in 2007).

Matheson was born in Allendale, New Jersey, to Norwegian immigrants Bertolf and Fanny, who divorced when he was 8. Matheson subsequently was raised in Brooklyn, New York by his mother. His early writing influences were the film Dracula, novels by Kenneth Roberts, and a poem he saw in the newspaper The Brooklyn Eagle, where at age 8 he would publish his first short story. After graduating from Brooklyn Technical High School, he served with the United States Army in Europe during World War II, an experience that formed the basis for his 1960 novel The Beardless Warriors. After returning home, he attended the Missouri School of Journalism at the University of Missouri, earning his Bachelor of Arts degree in 1949. Afterward, he moved to California.

For Hammer Film Productions he wrote the screenplay for Fanatic (1965; US title: Die! Die! My Darling!) based on the novel Nightmare by Anne Blaisdell, starring Tallulah Bankhead and Stefanie Powers; he also adapted for Hammer Dennis Wheatley's The Devil Rides Out (1968).

He wrote the Star Trek episode "The Enemy Within" (1966).

He adapted his 1971 short story "Duel" as a screenplay directed by a young Steven Spielberg, for the television film of the same name that year.

In 1973, Matheson earned an Edgar Award from the Mystery Writers of America for his teleplay for The Night Stalker, one of two TV movies written by Matheson and directed by Dan Curtis (the other was The Night Strangler, which preceded the TV series Kolchak: The Night Stalker). Matheson worked extensively with Curtis; the 1977 television movie Dead of Night features three stories written for the screen by Matheson — "Second Chance" (based on the story by Jack Finney); "No Such Thing as a Vampire" (based on Matheson's story of the same name); and "Bobby", an original script written for this omnibus movie by Matheson.

At the annual World Fantasy Conventions he won two judged, annual literary awards for particular works: World Fantasy Awards for Bid Time Return as the best novel of 1975 and Richard Matheson: Collected Stories as the best collection of 1989.

In the 1980s Matheson published the novel Earthbound, wrote several screenplays for the TV series Amazing Stories, and continued to publish short fiction.

Matheson received the World Fantasy Award for Life Achievement in 1984 and the Bram Stoker Award for Lifetime Achievement from the Horror Writers Association in 1991. The Science Fiction Hall of Fame inducted him in 2010.

Matheson published four western novels in this decade, plus the suspense novel Seven Steps to Midnight (1993) and the blackly comic locked-room mystery novel, Now You See It ..., aptly dedicated to Robert Bloch (1995).

Many previously unpublished novels by Matheson appeared late in his career, as did various collections of his work and previously unpublished screenplays. He also wrote new works, such as the suspense novel Hunted Past Reason (2002). and the children's illustrated fantasy Abu and the Seven Marvels.

The tribute anthology He is Legend was published by Gauntlet Press in 2009.

His first-written novel, Hunger and Thirst, was ignored by publishers for several decades before eventually being published in 2010, but his short story "Born of Man and Woman" was published in The Magazine of Fantasy & Science Fiction, Summer 1950, the new quarterly's third issue and attracted attention. It is the tale of a monstrous child chained by its parents in the cellar, cast as the creature's diary in poignantly non-idiomatic English. Later that year he placed stories in the first and third numbers of Galaxy Science Fiction, a new monthly. His first anthology of work was published in 1954. Between 1950 and 1971, he produced dozens of stories, frequently blending elements of the science fiction, horror, and fantasy genres.

Matheson died on June 23, 2013 at his home in Los Angeles, California, aged 87.

He also wrote several movies—the offbeat comedy and box-office flop Loose Cannons, the biopic The Dreamer of Oz (about L. Frank Baum), a segment of Rod Serling's Lost Classics, and Trilogy of Terror II. Short stories continued to flow from his pen, and he saw the adaptations by other hands of two more of his novels for the big screen—What Dreams May Come and A Stir of Echoes (as Stir of Echoes). In 1999, Matheson published a non-fiction work The Path, inspired by his interest in psychic phenomena.