We present 44 cases of congenital cholesteatoma of the middle ear. Twenty-one patients had a cholesteatoma located in the posterosuperior mesotympanum. This finding was in complete contrast to the commonly reported anterosuperior location, seen in only 2 cases in our study. The remaining 21 patients had a cholesteatoma involving either the entire mesotympanum and/or epitympanum. The posteriorly located congenital cholesteatoma might represent a completely different entity and originate from epithelial cell debris trapped in the posterior mesotympanum during development of the temporal bone. All but one patient were treated with a closed tympanoplasty. Eight patients underwent single stage surgery. A preplanned second stage procedure was performed in 33 patients, while 3 are presently awaiting the second stage. Residual disease was seen in 19 patients (57%) who had undergone second stage surgery. No patient has had recurrent disease this far. Thirty-eight patients (85%) had a preoperative air bone gap of 30 dB or more. Of the 33 patients evaluated for hearing results, 16 (48%) had a postoperative gap within 10 dB.