We report a case of right renal pelvic cancer with tumor thrombus in the inferior vena cava. A 65-year-old man with right flank abdominal pain and high fever was reffered to our hospital. Computed tomography showed right renal mass. Magnetic resonance imaging revealed tumor thrombus extending into the renal vein and the inferior vena cava. Preoperative diagnosis was renal cell carcinoma with vena caval thrombus. Radical nephrectomy with thrombectomy and lymphodenectomy was performed. Pathologic evaluation revealed transitional cell carcinoma with tumor thrombus into the vena cava. One course of M-VAC chemotherapy was added and he has been alive for 56 months without recurrence. A literature review of 15 cases of renal pelvic cancer with tumor thrombus in the vena cava in Japan revealed that 7 cases were diagnosed as renal cell carcinoma preoperatively.