Bilateral Congenital Cholesteatoma in a 13-Year-Old Boy

A congenital cholesteatoma is a cystic lesion composed of keratinizing squamous epithelium that arises behind an intact and normal-appearing tympanic membrane. It typically appears as a white, pearl-like mass and often presents with progressive conductive hearing loss. Although congenital cholesteatoma itself is rare, the bilateral form is extremely uncommon. Diagnosis relies on clinical examination, particularly otoscopy, and imaging may support the evaluation. Treatment is exclusively surgical to prevent complications and preserve hearing. We report the case of a 13-year-old boy diagnosed with bilateral congenital cholesteatoma, successfully managed with staged surgical excision.