A 22-year-old woman presented with three weeks history of intermittent left loin pain, on radiological evaluation by U\S and MRI revealed left renal pelvic mass, ureterorenoscopy and biopsy taken, but couldn't reveal definitive diagnosis other than presence of a malignant process. Left nephroureterctomy was performed. Grossly there is a polypoid mass attached to the upper pole of the kidney by stalk. Light microscopic examination and immunohistochemical staining confirm a diagnosis of Botryoid-type of embryonal rhabdomyosarcoma. Treatment and follow up to 1 year is mentioned. Reviewing the literature the presented case is the second of this tumor in adult renal pelvis.