Congenital cholesteatoma may originate at various sites in the temporal ban
e. For example, in the petrous apex, the cerebellopontine angle, the middle
ear cavity, the mastoid process or the external auditory canal. The least
common site being the mastoid process. We present two cases of congenital c
holesteatoma of the mastoid process, each presenting with different symptom
s and at different ages. Both patients underwent surgical treatment, which
confirmed the diagnosis and radiological findings.