Ameloblastic carcinoma (AC) is a rare malignant lesion with characteristic histologic features and clinical behavior that dictates a more aggressive surgical approach than that of a simple ameloblastoma. The tumor cells resemble the cells seen in ameloblastoma, but they show cytologic atypia. Direct extension of the tumor, lymph node involvement, and metastasis to various sites (frequently the lung) have been reported. Wide local excision is the treatment of choice. Regional lymph node dissection should be considered and performed selectively. Literature shows that radiotherapy and chemotherapy is of limited value for the treatment of AC. A case of AC of maxillary region is presented here. Clinical/histological characteristics of this tumor and current knowledge on the classification of odontogenic malignancies are also discussed.