--Introduction: Ameloblastic fibrosarcoma is a rare malignant odontogenic tumor. Over 50% reported cases have histological evidence of ameloblastic fibroma at the same site. The mortality rate of the tumor is 19% and its recurrence rate is 37%, which mandates a total resection along with long-term follow-up. Observation: We present the case of a 14-year-old male diagnosed with ameloblastic fibrosarcoma, who underwent multiple stages of treatments and was followed up over 8 years. The initial management consisted of a total tumor resection with chemoradiotherapy. Then, for bone, soft, mucosal, and dental restoration after resection, mandibular reconstruction with a fibula free flap, onlay iliac graft, iliac graft fixation, facial lipofilling treatments (two), vestibular deepening procedures (two), and implant-supported dental prosthesis were performed. Comments and conclusion: This is the first case reported in the literature for the global management of mandibular ameloblastic fibrosarcoma, from oncological treatment to functional and aesthetic long-term rehabilitation.
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