Osteosarcomas are rare, highly malignant, bone tumors deined by the presence of malignant mesenchymal cells producing osteoid or immature bone. Osteosarcomas of the jaws are extremely rare, representing about 7% of all osteosarcomas and 1% of all head and neck malignancies. An accurate diagnosis, usually facilitated by chemotherapy (CT), MRI and biopsy, is required in order to deine the stage of the disease and plane the adequate treatment. Aggressive surgical resection and advanced technique reconstruction are the mainstay of treatment, as the single most important factor for cure is radical resection. Clinical outcomes can be improved by a multimodal strategy combining surgery with neo-adjuvant and adjuvant chemotherapy in selected cases, and adjuvant radiotherapy in the absence of clear margins.