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 (MEC) accounts for 13% of all malignancies occurring in maxillary sinus.
Maxillary MEC have a worse prognosis than mandibular lesions and it should be followed-up for a longer period due to the possibility of late recurrence or regional metastasis. We report a rare case of mucoepidermoid carcinoma of maxillary sinus in a 16-year-old female with clear cell predominance.