RESUMO
This report presents an extremely rare tumor and provides information to deal with such tumors; intraosseous mucoepidermoid carcinoma. It's pathogenesis is still unknown and is rare in children. The conservative surgical procedures result to metastasis. An 11 year old girl was referred for a nonpainful swelling in maxillary region. Central mucoepidermoid carcinoma was diagnosed by clinical and paraclinical examinations. The lining of maxillary sinus was considered to be the source of the tumor. Hemimaxillectomy and adjuvant I radiotherapy was performed as treatment option and patient was reported to be in good health. Early diagnosis and radical resection with adjuvant radiotherapy offered the best chance for eradication of this tumor and that essential to prevent local recurrence and late distant methastasis