Ameloblastic carcinoma (AC) is a rare neoplasm of the maxillofacial district. It usually occurs in the posterior region of the jaw. In one third of cases, it occurs in the maxilla, usually in the posterior portion. From a review of international literature, we found 60 cases described. A 77-year-old patient presented to our division of maxillofacial surgery after the onset of a vegetans lesion of the maxilla. He had already been subjected to a first histologic examination that detected an AC. Surgery consisted of removal of a trapezoidal flap of the maxillary bone containing the mass. Histologic examination confirmed preoperative biopsy findings. Because of the low compliance, we did not plan for surgical reconstruction.Surgical resection is the treatment of choice for this kind of lesion, leaving at least a 2-cm free margin. Neoadjuvant radiotherapy may be useful for tumor debulking.