The author aims to describe the features of congenital cholesteatoma in adults and the value of the exclusive otoendoscopy in the surgical management. The congenital cholesteatoma is well known in children. This disease is uncommon in adults and is described here through a case-report in a 25-year-old patient. The forms localized to the upper posterior part of the tympanic cavity are the least common and can take the features of an acquired cholesteatoma. The surgical treatment is usually performed with otomicroscopy, with a canal wall-up tympanoplasty. In this case, the management was performed with a transmeatal approach with the exclusive otoendoscopic surgery. This procedure allows an access to anatomical areas hidden for the microscope. It belongs to a new surgical era in otology and is one of the minimal invasive surgical procedures of the future.
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