Purpose: We assess the benefit of retroperitoneal lymph node dissection for adult paratesticular rhabdomyosarcoma, Materials and Methods: A total of 19 adults with paratesticular rhabdomyosarcoma who underwent retroperitoneal lymph node dissection between 1980 and 1997 comprise our study population. Postoperative chemotherapy was administered in all patients, including cyclophosphamide, doxorubicin and vincristine with or without consolidation with ifosfamide and etoposide in 17, and vincristine, actinomycin D and cyclophosphamide in 2. Radiation therapy also was administered in 2 patients. Patients were classified into pathological and clinical groups according to the Intergroup Rhabdomyosarcoma Studies. Mean followup was 6.4 years (range 0.5 to 17.6). Results: A total of 17 patients (89%) were disease-free and 2 (1 in pathological group I and 1 in pathological group PT) died of disease that recurred outside of the retroperitoneum. Of 16 clinical group I patients 9 were in pathological group II. There were negative nodes in 2 of 3 clinical group LT patients. Thus, abdominal computerized tomography correctly staged only 8 of 19 patients (42%). Conclusions: Retroperitoneal lymph node dissection accurately stages paratesticular rhabdomyosarcoma and eliminates the need for abdominal radiotherapy in patients in pathological group II. Combined modality therapy with retroperitoneal lymph node dissection and postoperative chemotherapy achieves a high cure rate.