Background: Osteosarcoma is the most common primary malignancy and aggressive neoplasm of bone composed of spindle cells producing osteoid. The incidence of primary osteogenic sarcomas of the skull is about 1% to 2% of all skull tumors. Methods: We present an extraordinary case of osteosarcoma arising in the postero-temporal region of the skull. The clinical features, diagnosis and management are discussed. Results: A 34-year-old woman was operated in our department for mass, fixed to bone in the left postero-temporal area. Histopathology confirmed the diagnosis of osteogenic sarcoma. Conclusion: We review the literature of reported cases of primary osteogenic sarcomas of the skull to discuss the common clinical presentation, evaluation methods, and recommended treatment plans.