We report a case of paratesticular rhabdomyosarcoma in a 16-year-old male. The patient with a mass in the right scrotum was at first diagnosed with testicular tumor. Right orchiectomy revealed paratesticular rhabdomyosarcoma. Para-aortic lymph node metastasis was pointed out by abdominal computed tomography. Although he received multidrug chemotherapy consisting of vincristine, adriamycin, cyclophosphamide, cisplatin, and actinomycin-D (IRS-III regimen 35 and IRS-IV regimen) without radiotherapy, he died twenty-one months after the operation.