Congenital cholesteatoma of the ear is a capricious and challenging problem for the otologist. We present 21 cases managed at the Mayo Clinic from 1961 to 1983. In all but one unusual case, the cholesteatoma developed behind an intact tympanic membrane and there was no history of aural infections. Preoperative symptoms and signs were predominantly conductive deafness with either a normal tympanic membrane or an opaque, whitish appearance to the drum. Disease was located in the middle ear or in the middle ear and mastoid, except in one patient who had cholesteatoma extending into the petrous apex. Surgical management depended on the extent of the lesion, and recurrences were unusual, suggesting that congenital cholesteatoma may not be as aggressive as acquired cholesteatoma.