Rhabdomyosarcoma is a malignant tumor well known to urologists. These tumors arise from the genitourinary system in 20% to 25% of cases, most commonly from the bladder, prostate, vagina, and paratesticular region. This is the first reported case of a rhabdomyosarcoma arising from the ureter. The radiographic findings and ureteroscopic appearance of this tumor suggested a benign fibroepithelial polyp; however, a ureteroscopic biopsy and subsequent nephroureterectomy revealed an embryonal rhabdomyosarcoma.
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