The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.