Odontogenic carcinomas of the jaws are subclassified into malignant ameloblastoma, ameloblastic carcinoma and primary intraosseous carcinoma arising from within the bone. These may arise from residual islands of epithelium derived from dental lamina or epithelial lining of dental cysts. Ameloblastic carcinoma is extremely rare. An aggressive case of ameloblastic carcinoma occumng in a 59-year-old Malay man is presented. Wide excision of the primary lesion with radical neck dissection was carried out. He developed lung metastasis 4 months post-operatively. Despite chemotherapy upon discovery of lung metastasis, he expired 7 months following the initial diagnosis.