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Primary renal osteosarcoma is an uncommon disease, which, unlike its skeletal counterpart, presents mostly in adults, and is generally diagnosed late due to its non-specific features and intra-abdominal location. Even if the disease is localized at diagnosis, it follows an aggressive course despite radical surgery and adjuvant treatment. We report a case of renal osteosarcoma in a 65-year-old female, who developed regional recurrence, and lung and bone metastases soon after radical nephrectomy for localized disease. Chemotherapy was ineffective in controlling systemic disease.