OBJECTIVE: Cholesteatomas are cystic lesions lined with stratified squamous epithelium and filled with keratin with expansive capacity and bone lysis, usually located within the middle ear or other pneumatized areas of the temporal bone, may invade adjacent structures, and tend to relapse, even after resections. CASE REPORT: We describe a case of a 29-year-old woman with facial palsy and hearing loss secondary to left aggressive congenital cholesteatoma with significant erosion of the lateral and superior semicircular canals, the lateral wall of the attic, the promontory of the cochlea, and the ossicles, and with complete erosion of the anvil and stirrup body and the tegmen tympani. CONCLUSION: We emphasize the importance of early diagnosis and appropriate treatment of this disease in order to prevent complications related to its destructive, although insidious, behavior.