A patient with ameloblastoma of the mandible with histologically confirmed pulmonary metastases 9 years after onset of tumor is described. The effectiveness of three chemotherapeutic agents (cyclophosphamide, methotrexate, Adriamycin), each given alone intravenously, were evaluated. Marked symptomatic improvement was noted with Adriamycin therapy. Ten previous cases of metastatic ameloblastoma are reviewed. The incidence of metastases cannot be predicted on the basis of histology. Three commonly discussed modes of metastasis are via hematogenous and lymphatic routes and the unusual mechanism of aspiration of tumor cells.