Aim: Open- and closed-type congenital cholesteatoma in the middle ear can be found at the same time and an ear endoscope can be useful to identify and remove residual cholesteatoma during surgery. Background: Congenital cholesteatoma of the middle ear usually manifests as a single growth. Open- and closed-type congenital cholesteatomas occurring simultaneously in the middle ear on one side are extremely rare. In recent years, the use of endoscopes for middle ear surgery has been gradually increasing. Case description: This report describes the case of a 6-year-old boy in whom we observed both open-type and closed-type congenital cholesteatomas in the right middle ear. The closed-type congenital cholesteatoma extended from the malleus to the posterior wall of the tympanic cavity and had destroyed the long process of the incus and crura of the stapes. An open-type congenital cholesteatoma was observed on the medial side of the malleus, supratubal recess, and attic walls. The cholesteatoma was removed successfully via a transmeatal approach using an ear endoscope and surgical microscope. Using the ear endoscope, we could identify and completely remove the residues of cholesteatoma in the epitympanum, posterior tympanum, and supratubal recess. Conclusion: It is rare for both open-type and closed-type cholesteatomas to exist simultaneously in the middle ear, as in this case. Although the cholesteatomas were more extensive than the single lesion that usually occurs, both could be removed successfully via a transmeatal approach using a surgical microscope and an ear endoscope. Clinical significance: It is important to keep in mind the possibility of simultaneous open-type and closed-type congenital cholesteatomas in the middle ear, and the use of endoscopes can reduce the extent of the surgery and help identify residual cholesteatoma.