The development of Wilms tumor in a patient with Prader-Willi syndrome prompted us to determine the parental origin of the genes implicated in both disorders because of the sex-specific parent-of-origin effects previously demonstrated for both conditions. A paternal chromosome 15q11-q13 deletion was demonstrated, but no changes were demonstrated in a limited analysis of chromosome 11p, which harbors two Wilms tumor suppressor genes, WT1 and WT2.