A 20-year-old male presented with an osteosarcoma in the right parieto-occipital bone occurring as a painless occipital lump which had rapidly enlarged in the 6 months prior to admission. The neuroimaging appearance resembled intraosseous meningioma. Gross total resection of the tumor was achieved. The final histological diagnosis was osteosarcoma. Osteosarcomas of craniofacial region have a better prognosis than those of the skeletal bones, and distant metastasis is rare. Local recurrence is the most significant factor contributing to poor outcome. Complete excision with negative margins is the key to a better outcome. Adjuvant therapy may be an option in cases of incomplete excision. Advances in target chemotherapy may diminish the significant morbidity associated with these lesions.