BACKGROUND Congenital cholesteatoma is thought to be caused by inadequate folding of the epidermoid formation inside the middle ear cleft. During development of the middle ear mucosa, stratified squamous epithelium accumulates in the embryonic life. Its typical appearance is a "pearl" beneath the anterosuperior quadrant of the tympanic membrane. METHODS We report 28 years-old case with congenital cholesteatoma in the posterosuperior quadrant of middle ear cavity. The main complaint was the hearing loss which had developed slowly over several years. CONCLUSIONS The case was surgically treated. Postoperative hearing result was satisfactory. CONCLUSIONS Congenital cholesteatoma may occur in atypical locations and ages. Many authors prefer canal wall down tympanomastoidectomy. But it can also be treated successfully by intact canal wall tympanomastoidectomy with good hearing results.