Cholesteatoma refers to the presence of keratinizing squamous epitheli
um in the middle ear, mastoid, or petrous bone. This histologically be
nign lesion can erode bone, causing hearing loss, disequilibrium, faci
al nerve palsy, venous thrombosis, and intracranial infection. There a
re two main types of cholesteatoma: congenital and acquired. Cholestea
toma in children can pose diagnostic and therapeutic challenges for th
e clinician. The pathophysiology, clinical presentation, diagnostic ev
aluation, and management of children with these lesions are discussed.