We studied 48 patients (48 ears) with congenital cholesteatoma who underwent surgery at our department from 1979 to 2000, and investigated symptoms at initial onset, tympanic membrane findings, cholesteatoma configuration and site, type of surgical procedure, and surgical outcome. Patients were from 2 to 62 years old (mean: 16.7 years), with 60.4% aged 15 years or younger. The symptom at initial onset was hearing loss in most (58.2%). Hearing loss was the main symptom in all with open type cholesteatoma, and most of these patients had normal tympanic membrane findings. The cholesteatoma was located mainly in the superior posterior portion of the tympanic cavity in many patients. The site of involvement was the tympanic cavity in 12 (25.0%), mastoid cavity in 2 (4.2%) and the petrous apex in 1 (2.1%). In many of (31 ears, 64.6%), the cholesteatoma was advanced and extended from the tympanic cavity to the mastoid antrum. For 23 of the 48 ears, treatment was completed in one operation. The remaining 25 ears required staged surgery. Loss of the structure of the upper part of the stapes was seen in 58.3% of patients, so most underwent type IV ossiculoplasty, with types III and I next most common.