The authors present three cases of congenital cholesteatoma in children. In two cases primary surgery was curative, in third patient one year after first surgery the residual cholesteatoma was diagnosed, and second surgery was performed. Finally in all cases good anatomical and functional effect was obtained. The natural history of congenital cholesteatoma, as well as surgical techniques which can be applied depending on type and location of congenital cholesteatoma are discussed. The authors emphasise the importance of early recognition of the disease. Exploratory tympanotomy, or CT scan should be applied in all cases where suspected changes are observed during otoscopy.