A 68-year-old man underwent left side simple nephrectomy for symptomatic severe hydronephrosis with decreased function due to a renal stone. Because of severe adhesion around the kidney, the renal pelvic wall was torn during the operation. Pathological diagnosis was papillary adenocarcinoma of the renal pelvis with positive staining for carbohydrate antigen 125 (CA125) and carbohydrate antigen 19 9 (CA19-9). Retrospective analysis of preoperative blood sample showed a high level of CA125 and CA19-9. Four-cycle adjuvant chemotherapy with paclitaxel/carboplatin (TJ regimen) was performed. However, local recurrence developed 1 month after the termination of chemotherapy. Although papillary adenocarcinoma of the renal pelvis is extremely rare, the possibility of renal pelvic tumor should be kept in mind for patients who have a long-standing renal stone and hydronephrosis with irregularity at the renal pelvic wall. CA125 and CA19-9 can be useful markers for upper urinary tract tumor.