We have described an adult with Wilms' tumor treated with surgery, radiotherapy, and chemotherapy. Although the patient responded initially, he died 17 months after diagnosis. A review of the literature in English disclosed only six other adults with Wilms' tumor similarly treated. Only two were long-term survivors, both treated with combination chemotherapy and radiation therapy. Adult Wilms' tumor is rare and carries a more pessimistic prognosis than in children, but it is responsive to radiation and combination chemotherapy.