Congenital cholesteatoma develops from the squamousepithelium that is located at middle ear, petrous boneand mastoid bone during embryological development ofmiddle ear. Congenital cholesteatoma forms the 2-5% ofall cholesteatomas. In this article, a 5 years old boy withbilateral -probably congenital- diffuse cholesteatoma andmastoid fistulas is presented. Our case is special dueto his age and severe destructive pattern. Patient wastreated successfully by bilateral radical mastoidectomyoperation.Key words: Congenital, cholesteatoma, mastoiditi