Neurofibroma is a benign tumor of the connective tissue of the peripheral nerves, developed mainly at the endoneurium. The most common localizations are the extremities of the limbs and the head and neck region. Neurofibromas are often associated with neurofibromatosis type 1. An isolated localization in the auditory canal is exceptional. We report the case of a 45-year-old female patient presenting with a neurofibroma of the auditory canal which had previously caused hearing loss. Examination revealed a tissue mass firm, painless and covered with normal skin obstructing the external auditory canal. The patient’s skin examination revealed no café-au-lait spots. A CT scan of the ear showed a hypodense tissue mass. Surgical removal via the ear canal gave good results. In conclusion, an isolated neurofibroma of the external auditory canal is a rare benign tumor with a good prognosis. The surgical approach depends on the exact location of the mass and the surgeon’s experience.
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