The incidence in Wilms' tumor patients of constitutional chromosome defects other than those involving the short arm of chromosome 11 was examined using data on 5,854 patients registered in the National Wilms Tumor Study. Trisomy 18 and Turner syndromes were found to occur at higher rates than expected based on chromosome surveys of newborns. Four patients were reported to have trisomy 18; all of these patients were over 5 years of age at the time of diagnosis of Wilms' tumor and none survived longer than six months. Four patients were reported to have Turner syndrome; these patients are currently doing well and have all survived at least 3 years following diagnosis. Two of the Turner patients and one of the trisomy 18 patients had horse-shoe kidneys; we speculate that this renal anomaly may contribute to the higher rates of tumor in these patients. No clear pattern was found among patients with other chromosome defects, although two patients had defects involving 2q37.