Craniofacial osteosarcoma is a relatively rare entity comprising about 6.5-7% of all osteosarcomas, whereas it is reported to be one of the most common malignancies of long bones. We present a case of chondroblastic variant of osteosarcoma (COS) of the maxilla in an 18 year-old-girl. She was treated with a subtotal maxillectomy and later referred for adjuvant chemotherapy. The relevant review of literature with regard to clinical, radiographic findings, histopathological similarities to Chondrosarcoma (CS), role of Immunohistochemistry (IHC) and management modalities of Osteosarcoma of jaw bones (OSJ) in comparison with Osteosarcoma of the long bones (OSL) are being presented in this report.

SDC/MISC/2013/239.