A 38-year-old male presented with an extremely rare primary osteogenic sarcoma, unassociated with Paget''s disease or late effects of radiation, involving the sella and sphenoid sinus region. Complete excision of the tumor was achieved through an extended frontobasal approach. Postoperatively, six cycles of combination chemotherapy (adriamycin, ifosphamide, and cisplatin) followed by a total of 55 Gy local radiotherapy in 33 fractions was given. Primary osteogenic sarcoma should be considered in the differential diagnosis of the central skull base tumors. Osteogenic sarcoma, in general, has a bad prognosis, and should be managed aggressively with multimodality treatment including gross total surgical resection, combination chemotherapy, and radiotherapy.