Material and methods A case of ameloblastic carcinoma of the maxilla arising in a 90-year-old patient is presented along with a review of 65 other cases of the international literature. Results The median age was 44 years with a predominance of men (42/66). The maxilla was concerned in almost one third of cases (21/66). Twenty patients died of disease after a median time of 60 months. Fifteen patients died with metastatic spread in the lung, brain, or bones; the others were due to a local recurrence. The specific survival rate was 68.7% at 5 years. Conclusion Ameloblastic carcinoma is a rare entity of odontogenic tumors that exhibits malignant histologic features in the primary site. Specific mortality, estimated at 31.3% at 5 years, was generally due to a metastatic spread.