Ameloblastoma is an aggressive benign tumor with frequent local recurrences. Although histologically benign, it occasionally metastasizes to many organs, most commonly to the lungs. The metastasis develops after multiple recurrences and many unsuccessful attempts at removal of the tumor. When distant metastasis occurs, the prognosis is poor and there is no effective treatment. A case of metastatic ameloblastoma of the mandible and maxilla is reported which was treated with pulmonary metastasectomy. The treatment options are discussed in relation to the literature.