Bilateral congenital cholesteatomas are rare entities. Nine cases have been previously described in the literature. Many different etiologies for the development of congenital cholesteatoma have been proposed. The case of a five-year-old boy with bilateral congenital cholesteatoma is discussed. A lesion of the left ear was apparent clinically. However, the right-sided lesion was silent and was demonstrated only by radiologic exam. Theories of pathogenesis are reviewed.