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Introduction: Mucoepidermoid carcinoma represents one of the most common malignant salivary gland tumors.However, the sclerosing morphologic variant is extremely rare with only 23 reported cases in the English-language literature since it was discovered in 1987. Case Report: Herein, we describe another case that was diagnosed in a 25-year-old woman presenting with a posterior auricular mass, as well as a review of the literature, which demonstrates that this is an extremely rare malignancy with no strict protocol for treatment. Conclusion: Pathologists must be aware of recognizing low grade sclerosing mucoepidermoid carcinoma which has metastatic potential and is frequently misdiagnosed as a benign lesion.