Extrarenal Wilms' tumor is rare and has been reported in the literature principally as case reports. Its clinical presentation varies according to the extrarenal localization. The procedures utilized to determine the size of the primary tumor, regional node involvement and the presence of distant metastasis are similar to those utilized in Wilms' tumor of the kidney. Our results demonstrate the utility of chemotherapy; the cytostatic agents utilized appear to be as effective as in Wilms' tumor of the kidney. In our view, radiotherapy should be reserved for the large unresectable residual tumor mass and for distant metastasis.