Renal sarcomas are relatively uncommon among all renal malignancies. A renal rhabdomyosarcoma almost always manifests as a renal parenchymal tumor. A 77-year-old female was diagnosed with a right renal pelvic tumor, measuring 2×2×1.8 cm, after the workup for hematuria. She received a nephroureterectomy with bladder cuff excision under the preliminary impression of urothelial cancer. This is a rare presentation of a renal rhabdomyosarcoma. She remained free of recurrence 2 years after receiving only surgical treatment.