Malignant tumors of the paranasal sinus are uncommon, constituting less than 1% of all malignancies and 3% of all head and neck cancers. Nonsquamous cancers of the maxillary sinus are even rarer as is evident from the limited data available on the clinical characteristics and outcomes. Mucoepidermoid carcinoma accounts for 13% of all malignancies occurring in maxillary sinus. We report a rare case of high-grade mucoepidermoid carcinoma of maxillary sinus in a 45-year-old female along with review of the literature.