Keywords: ameloblastic carcinoma, odontogenic tumors, histopathological diagnosis

Abstract: Ameloblastic carcinoma is an unusual tumor. There have been a total of 36 cases of ameloblastic carcinoma in the English literature to date. Of these, only 12 cases have occurred in the maxilla. The WHO classification of odontogenic carcinoma refers the term malignant ameloblastoma as a neoplasm with features of an ameloblastoma in the primary and metastatic growth. Slootweg and Muller (1984), in addition, included the ameloblastic carcinoma as the primary tumor with malignant transformation, regardless of its metastatic potential. We report a rare case of extensive maxillary ameloblastic carcinoma with extensive cranial invasion. The surgical treatment was palliative due to the intracranial invasion of the tumor. There was great difficult to establish the histopathological diferencial diagnosis among squamous cell carcinoma, inverted papilloma, ameloblastoma and ameloblastic carcinoma.