Primary embryonal rhabdomyosarcomas of the kidney are extremely rare, especially in adults. The presented case, a 32-year-old female with a background of autosomal polycystic kidney disease, was initially referred with a left hemorrhagic renal cyst. Despite angioembolization, she eventually underwent radical nephrectomy which revealed the diagnosis of embryonal rhabdomyosarcoma. The diagnosis and presentation of this case is unique as she presented with hemorrhagic renal cyst. Adult renal rhabdomyosarcoma has a poor prognosis, as shown by other reported cases. Unfortunately, She passed away 88 days post operatively due to disease progression.