Ameloblastic carcinoma is an extremely rare, malignant epithelial tumor of the jaws with a poor prognosis. The most common site of occurrence is the posterior mandible. Clinically, it is very aggressive and has potential for extensive local destruction. Majority of the cases arise de novo (primary type), but a few cases arise from a pre-existing ameloblastoma (secondary type). Early diagnosis and wide local excision on the primary site is the treatment of choice. The risk of malignant transformation should always be considered when a classic ameloblastoma is diagnosed and the prompt definitive management of an ameloblastoma is essential to eliminate this risk. Finally, meticulous, long-term follow-up is mandatory because recurrence and metastasis in the lung and regional lymph nodes have been reported.