We report a case of sarcomatoid renal cell carcinoma in a 30-year-old woman. The pathological findings indicated renal cell carcinoma (solid sarcomatoid and pleomorphic type) similar to malignant rhabdoid tumor of kidney (MRTK). We treated her with combination chemotherapy (vincristine, adriamycin, cyclophosphamide and actinomycin D) and local irradiation after nephrectomy. The number of bone metastatic foci increased and brain metastasis occurred. However, irradiation was effective on each focus. She died 8 months after the operation caused by brain metastasis. We conclude that sarcomatoid renal cancer is highly malignant and chemotherapy-resistant, but radiation may be partially effective.