Cholesteatomas are abnormal collections of squamous epithelium and keratin debris that usually involve the middle ear and mastoid. Although histologically benign, they have the ability to expand and destroy bone. Cholesteatomas are treated surgically. The success of such surgery is highly dependent on the extent of the lesion. This article presents information and images to aid the general pediatrician in the early recognition of cholesteatomas, both congenital and acquired, in hopes of improving the outcome for children with this treatable disorder.