Sclerosing mucoepidermoid carcinoma (SMEC) is an extremely rare variant of mucoepidermoid carcinoma with unique histologic features. To the best of our knowledge, only 14 cases of salivary gland SMEC have been previously reported in the English-language literature since it was discovered in 1987. We report a new case of salivary gland SMEC, and we review the literature. Because of SMEC's distinctive histologic features and rarity, its diagnosis can be difficult. Moreover, no clear treatment strategy has emerged with regard to adjuvant postoperative radiotherapy. We attempt to elucidate a natural course of this disease, and we propose a conservative approach to postoperative management.