Four cases which was suspected of congenital cholesteatoma were treated in the past 15 years. The age of the patients ranged from 4 to 17 years. In all cases, the cholesteatoma arose in the middle ear. In one case the cholesteatoma extended into the mastoid cavity. Tympanoplasty of colummella type III was indicated. Postoperative hearing gain was from 20 to 30dB. Although the incidence is low, otolaryngologists must be aware of congenital cholesteatoma when treating conductive hearing loss with a normal ear drum.