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Abstract 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.