We report a case of bilateral congenital cholesteatoma in a 6-year-old boy. Cholesteatoma was present in both ears around the tympanic isthmus (the only open passage from the tympanic cavity to the attic), extending to behind the horizontal portion of the facial nerve. This patient underwent a total of three canal wall up operations on each side to remove the cholesteatoma completely and improve hearing. This case fulfills the criteria proposed by Derlacki and Clemis in 1965. Bilateral congenital cholesteatoma is a rare condition, but the incidence of congenital cholesteatoma in children has increased recently for the following reasons: Reassessment of the criteria, the introduction of computed tomography, and increased awareness of congenital cholesteatoma. When operating on patients with bilateral cholesteatoma, the best method for preservation of hearing should be chosen. We discuss these problems in the present report.