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Objective: Case report of a rare adenoid cystic carcinoma of the external auditory canal. Observation: A seventy-year-old patient who presented with an external auditory canal tumor, the biopsy of which revealed an adenoid cystic carcinoma of the external auditory canal. The CT scan did not show any local or regional spreading. The patient underwent a surgical procedure followed by radiotherapy, given the aggressiveness of the tumor. The evolution was favorable. Conclusion: Adenoid cystic carcinoma of the external auditory canal is rare but a high potential for recurrence exists, which justifies aggressive treatment.