Adult Wilms' tumor is a rare and aggressive malignancy. Despite multimodality therapy for all stages of disease, the majority of patients develop fatal metastases. Little information is available on effective salvage therapy. The current report describes a 38-year-old man who had Stage I disease. Pulmonary metastases developed after radical nephrectomy and tumor bed irradiation while receiving adjuvant chemotherapy. He achieved a complete remission lasting 8 months with a combination chemotherapy regimen of cyclophosphamide, vinblastine, dactinomycin, and cisplatin. After isolated pulmonary relapse, he received whole-lung irradiation and remained disease-free 4.5 years after diagnosis. The previously reported results of therapy in metastatic or unresectable adult Wilms' tumor are reviewed, and the current case of effective salvage therapy is documented.