Thirteen reported cases of renal pelvic sarcomatoid carcinoma have been documented since 1961, and all patients presented with metastatic disease or advanced renal parenchyma involvement. The mean survival was shorter than 9 months, and adjuvant therapy appeared to offer no benefit. We present the case of a 61-year-old man with gross hematuria and a large filling defect on computed tomography excretory urography. Surgical pathologic examination after laparoscopic nephroureterectomy was consistent with renal pelvis-confined sarcomatoid carcinoma of transitional cell origin. Adjuvant therapy was not given secondary to the organ-confined nature of disease. The patient was without recurrence for more than 1 year.