A 63-year-old man had an unresectable metastatic Wilms tumor in the right kidney. Radiotherapy to the abdomen and chest wall as well as chemotherapy in the form of actinomycin D, vincristine, and cyclophosphamide was given. The tumor was radioresponsive but failed to respond to chemotherapy. The literature was reviewed. Other reported cases of Wilms tumor in adults treated with chemotherapy that is usually given to children are few, but the results are mostly disappointing.