Levin studied at New York University, where he majored in philosophy and English. After that, he wrote training films and scripts for television. He wrote his first novel, A Kiss Before Dying, when he was 22 years old.

Levin is a versatile writer whose work includes thrillers and comedy. He wrote the play No Time for Sergeants (adapted from Mac Hyman's novel), which later became a popular film that launched the career of Andy Griffith. Levin's best known play is Deathtrap, which is still the longest running play on Broadway. In 1982, it was made into a film starring Christopher Reeve and Michael Caine.

Levin's best known novel is Rosemary's Baby, a horrifying tale of satanism and the occult. It was made into a film as were four of his other novels, A Kiss Before Dying (twice), The Boys from Brazil, The Stepford Wives (twice) and Sliver.

Stephen King has described Ira Levin as "the Swiss watchmaker of suspense novels, he makes what the rest of us do look like cheap watchmakers in drugstores." Chuck Palahniuk, in Stranger Than Fiction: True Stories, calls Levin's writing "a smart, updated version of the kind of folksy legends that cultures have always used."