Paratesticular rhabdomyosarcoma is a rare malignant disease most commonly seen in infants and children. In contrast with rhabdomyosarcomas in sites other than the genitourinary tract, paratesticular rhabdomyosarcomas tend to metastasize via a lymphatic route. This influences the therapeutic approach in which surgery, chemotherapy and, in selected cases, radiation therapy play an essential role. On hand of 7 of our own patients, uniformly treated at one institution, a review of the current approach of paratesticular rhabdomyosarcomas with excellent therapeutic results is given.