Abstract
Ameloblastic fibrosarcoma is an extremely rare odontogenic sarcoma, which consists of a benign epithelial and a malignant mesenchymal component. This rare tumor occurs de novo or from a pre-existing ameloblastic fibroma. We report a case of a 31-year-old male patient who developed a lung metastasis fibrosarcoma secondary to ameloblastic fibrosarcoma arising on a pre-existing mandibular ameloblastic fibroma. A thorough understanding of the radiological and histological aspects of mandibular tumors is essential to establish an accurate diagnosis and rule out other common benign and malignant odontogenic tumors that might display microscopic similarities. An appropriate management, including total surgical excision with safe margins, represents the preferred treatment of ameloblastic fibroma. Close monitoring is indicated to reduce the recurrence rate.