[Congenital cholesteatoma of the mastoid]. / Le cholestéatome congenital de la mastoïde.

UNLABELLED: The congenital cholesteatoma of the mastoid is rare and asymptomatic. CASE REPORT: We report a case of a young boy (5 year old) who presented mastoiditis, post auricular fistulisation and polyp of the external auditory canal. The mastoidectomy was performed and a cholesteatoma of the mastoid and the antrum was removed. No cholesteatoma was found in the tympanic cavity and the ossicular chain was normal. This surgery implies the complete removal of cholesteatoma and its matrix. One year after the patient showed no recurrency. CONCLUSION: The silent evolution of the congenital cholesteatoma explains the delay of its diagnosis and the risk of mastoiditis.