IntroductionPleomorphic adenoma accounts for 65 percent of all salivary gland tumors. It has been identified in several anatomical regions, but pleomorphic adenoma arising in the ear canal, first described in 1951, is extremely rare.Case presentationA 40-year-old Japanese man’s left ear canal was obstructed by a pleomorphic adenoma that caused mild conductive hearing loss. The tumor was resected and he remains disease-free two years after surgery.ConclusionsPleomorphic adenoma usually arises from a major and minor salivary gland, but pleomorphic adenoma of the ear canal is derived from the ceruminous gland. We discuss the present case and 37 other case reports in our effort to clarify the clinical features and the course of pleomorphic adenoma in the ear canal.

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