Primary renal osteosarcoma is an exceedingly rare tumor with poor prognosis. It tends to spread to distant sites and has a low survival rate. In this report, we present a case of a 43‐year‐old female patient presented with flank pain, gross hematuria, weakness, and weight loss. The patient was diagnosed with primary renal osteogenic sarcoma. This case report aimed to describe the clinical and pathological features, pathogenesis, and treatment modalities of this neoplasm.

Primary renal osteosarcoma is exceedingly rare tumor with poor prognosis. It has tendency to spread to distant sites . we report the case of a 43-year-old female who presented with flank pain, gross hematuria, weakness and weight loss and was treated with diagnosis of primary renal osteogenic sarcoma