ABSTRACT
Ameloblastoma is the most common benign odontogenic tumor of jaw, whereas ameloblastic carcinoma is extremely rare, aggressive odontogenic neoplasm of mandible and maxilla. Ameloblastic carcinoma is odontogenic malignancy that combines the histological features of ameloblastoma with cytological atypia regardless of metastasis. The clinical course is described as typically aggressive, with extensive local destruction and distant metastatic spread. Ameloblastic carcinoma has been found mostly in the mandible; however, its maxilla lesion has been reported less than 40 cases in the literature. We experienced a case of ameloblastic carcinoma in the maxillary sinus in a 43-year-old male presenting with right facial swelling. No evidence of recurrence was noted at a 5-year follow-up.