The authors present the case-history of a patient with congenital cholesteatoma in the pontocerebellar angle. In the clinical picture predominated neuralgia of the trigeminal nerve. The lesion was extirpated by a translabyrinthine approach. The conclusion of the histological examination was cholesteatoma. In the discussion the authors deal in more detail with the problem with special emphasis on the clinical picture, importance of imaging methods - CT and NMR - and the strategy of surgical treatment.

        Key words: epidermoid, congenital cholesteatoma in the pontocerebellar angle.