Congenital cholesteatoma in the ear is a challenging problem for the otolaryngologists. Fourteen cases of congenital cholesteatoma managed surgically at the Seoul National University Hospital from January of 1980 through March of 1994 were reviewed. Six cases in which the lesion was located in the middle ear showed conductive hearing loss and normal tympanic membrane, and hearing gain was present in most patients without recurrence of disease postopertively. But 8 cases in which the disease was located at the perigeniculate area, the petrous area, or the cerebellopontine angle showed facial palsy, severe hearing loss, vertigo, tinnitus, or other symptoms of cranial nerve dysfunction. Postoperatively, hearing gain was absent in these cases, and complications or sequelae were present. And there were recurrence or residue in 2 cases.