摘要Sarcomatoid transitional cell carcinoma of the renal pelvis is a rare neoplasm with only 14 well-illustrated examples reported previously. Duplex kidney is the most common congenital abnormality of the urinary tract, with an incidence of around 2%. Neoplasia of the renal pelvis in duplex kidney is rare. We reported a case whose sarcomatoid carcinoma originated from the upper portion of the duplicated renal pelvis with hydronephrosis, and total nephroureterectomy with bladder cuff excision surgery of both renal units was carried out. Because of the rare nature of renal pelvic sarcomatoid carcinoma and its apparent lack of response to adjuvant therapy, it is essential to do early diagnosis and early radical surgery to improve survival. It is important to stress the need for frequent and diligent monitoring or treating complex duplex kidney with hydronephrosis of either moiety in case of a risk of having neoplasias.