BACKGROUND: Ameloblastoma is a rare benign odontogenic tumor with locally 
aggressive behavior and a high recurrence rate. When metastases occur, which are 
uncommon, lungs constitute the most frequent site involved. Malignant 
ameloblastomas are different from ameloblastic carcinomas. Malignant 
ameloblastomas are tumors considered metastatic despite the appearance of 
well-differentiated or benign histology, while ameloblastic carcinomas are 
histologically malignant in both primary and metastatic sites.
CASE PRESENTATION: A 24-year-old Moroccan man presented a malignant 
ameloblastoma of the mandible. The tumor was entirely resected. Five years 
later, a local recurrence occurred. Our patient was treated by exclusive 
radiotherapy with persistence of a residual disease. After two years he 
developed multiple lung metastases. Our patient received a combination 
chemotherapy using doxorubicin and cisplatin.
CONCLUSION: Less than 50 cases of ameloblastoma with metastases have been 
reported. There is still no standard treatment for metastatic ameloblastoma. 
Only through continuous reporting of such cases will clinicians be able to draw 
an optimal strategy for management of this pathology.