From 1978 to 1993, 59 patients (60 ears) with congenital middle ear cholesteatoma were treated at the House Ear Clinic. The median patient age at presentation was 5 years, and the period of postoperative follow-up was 4.8 years. An intact canal wall was maintained in 58 of 60 cases and a closed middle ear space in all cases. In 12 operations, lateral graft tympanoplasty eradicated the cholesteatoma in one stage; 32 patients required a second-stage surgery to rule out recurrence, and the remaining 16 cases required three or more operations to eradicate disease and reconstruct the hearing mechanism. Thirty-five (63%) of 56 patients had a postoperative air-conduction threshold pure-tone average (PTA) within 10 dB of the best bone-conduction PTA; 91% were within 20 dB. Average speech reception threshold improved from 32 dB hearing level (HL) preoperatively to 20 dB HL postoperatively.