Learning Objectives: To present the features of congenital cholesteatoma.

Design: Case series

Patients and Methods: Ten patients were included in the study. The diagnosis of congenital cholesteatoma was based on previous history that excluded tympanic membrane perforation, otorreha, or otologic procedure, an intact tympanic membrane on otomicroscopic examination and a identified cholesteatoma at the time of surgical procedures (tympanotomy, atticotomy, tympanomastoidectomy).

Results: Six of the 10 patients had lesions isolated to the anterosuperior quadrant of the tympanum, the other had more extensive cholesteatoma that involve posterior part of the tympanic cavity and mastoid. Three of the patients underwent surgery for recidivism(none were from isolated anterior lesions). One of these patients was referred at the time of recurrence, one had known residual cholesteatoma, and one had recurrence.

Conclusion: Clinical and surgical findings suggest that congenital cholesteatoma showed various characteristics depending on the location and stage of development.