A 63-year-old man visited our hospital with body weight loss. Laboratory examination revealed a high serum level of carbohydrate antigen 19-9 (CA19-9) and LDH. There were no abnormal findings in the gastrointestinal tract. Enhanced abdominal computed tomography (CT) revealed a renal tumor, 5×3 cm in diameter, in the right lower pole and multiple lymph node swelling. The right renal tumor was not a typical renal cell carcinoma, so we considered the presence of bellini duct carcinoma and renal pelvis carcinoma, we performed right nephroureterectomy. Histopathological diagnosis was urothelial carcinoma with glandular differentiation of the renal pelvis. Post operation chemotherapy with GC (gemcitabine/cisplatin: 3-cycle), MVAC (methotrexate/vinblastine/doxorubicin/cisplatin: 1-cycle), TS-1 + CBDCA (tegafur-gimeracil-oteracil potassium/carboplatin: 3-cycle) was performed for lymph node metastasis, but he died of cachexia 18 months after operation.