A 61-year-old man presented with a right renal mass with a vena caval thrombus on computed tomography that was consistent with renal cell carcinoma. The results of routine laboratory examinations and urinalysis were within normal limits. Preoperative planning was critical owing to the presence of the vena caval thrombus. A radical nephrectomy, vena caval thrombectomy, and regional lymphadenectomy were done. The pathologic report was consistent with a high-grade, invasive urothelial carcinoma, with sarcomatoid differentiation involving the renal vein and inferior vena cava (Stage IV, T4N0M0). Thus, this was a rare case of upper tract urothelial carcinoma. Adjuvant chemotherapy with the methotrexate, vinblastine, doxorubicin, cisplatinum regimen is scheduled. To our knowledge, this is the first report in Korea of upper tract urothelial carcinoma of the sarcomatoid type with a vena caval thrombus.