Denys-Drash syndrome and WAGR syndrome are due to alterations of Wilms Tumor 1 (WT1) [5], and mutations in tumor protein 53 (TP53) [6, 7] and beta-catenin (CTNNB1) [8, 9] are associated with WT susceptibility. The gene discussed is TP53; the disease is Denys-Drash syndrome.