Forty-three patients with congenital middle ear cholesteatoma have been described since the first case in 1953. In these patients ten cholesteatomas were confined to the anterior mesotympanum in young children who had no ossicular damage. Involvement of the posterior mesotympanum (8 ears), meso and epitympanum (21 ears), and antrum and middle ear (7 ears) was associated with an 81% incidence of ossicular erosion in addition to other abnormalities. Anterior mesotympanic cholesteatomas were readily identified by the appearance of a white mass behind a translucent drum. The other congenital cholesteatomas had a more varied appearance with a mass and a whitish appearance each noted in about half. In almost half there was a bulging of the TM. Two new cases are reported: a 4-year-old with an extensive epi and mesotympanic cholesteatoma initially misdiagnosed as non-suppurative otitis media and a 23-year-old with the third reported case of bilateral congenital cholesteatoma.