A52-year-oldmanconsultedhisdoctorwithcomplaintsoflowerabdominalpainfor1year.Blood andurineanalysesshowedurinaryoccultbloodbutnoanemiaorelevatedtumormarkers.Computedto-mographyandmagneticresonanceimagingrevealeda75×62 mmretroperitonealtumorwithnometastasis.Thetumorwasincontactwiththeleftcommoniliacarteryandtheleftureterwascompressed,resultinginhydronephrosis.Thepatientwasreferredtoourhospitalaftertheplacementofaleftureteral stent.Biopsywasdifficultduetotumorlocalization;therefore,retroperitonealtumorresectionwasperformed.Histopathologyrevealedapleomorphicrhabdomyosarcoma.Therewasalocalizedrecurrenceafter 3 years. We performed tumor resection and postoperative radiation therapy at 50 Gy. The clinical coursewasfavorable,withnorecurrenceormetastasisfor6yearssincethefirstsurgery.