Mucoepidermoid carcinoma is the most common malignant salivary gland neoplasm. Its sclerosing morphologic variant, however, is an extremely rare entity. Only 7 cases have been reported in the literature, and only 2 of those reports have discussed this unusual malignancy in detail. This report describes a case involving a 57-year-old woman with an intermediate-grade sclerosing mucoepidermoid carcinoma of the parotid gland. She underwent superficial parotidectomy and radiation therapy with excellent results. Diagnosis and options for treatment are discussed, as well as a review of the literature, which demonstrates that this is an extremely rare malignancy with no strict protocol for treatment.