A cholesteatoma originating in the external auditory canal is a rare finding. We report 2 cases illustrating its typical localization in the inferior part of the auditory canal and its marked osteolytic character. The extension of such a cholesteatoma in the depth of the temporal bone is often larger than suggested by clinical examination. Since the purpose of treatment is to achieve the complete eradication of the epidermic matrix, surgical treatment is required in most cases. We discuss herein the follow-up strategies, keeping in mind that local recurrence and bilateral disease are not uncommon.