His first independent work, The Unstrung Harp, was published in 1953. He also published under various pen names, some of which were anagrams of his first and last names, such as Ogdred Weary, Dogear Wryde, Ms. Regera Dowdy, Eduard Blutig and dozens more.

Gorey's illustrated (and sometimes wordless) books, with their vaguely ominous air and ostensibly Victorian and Edwardian settings, have long had a cult following. He made a notable impact on the world of theater with his designs for the 1977 Broadway revival of Dracula, for which he won the Tony Award for Best Costume Design and was nominated for the Tony Award for Best Scenic Design.