To date, five Wilms tumor susceptibility loci have been well characterized, including Wilms tumor gene 1 (WT1), Wilms tumor gene on the X chromosome (WTX), catenin beta 1 (CTNNB1), tumor protein 53 (TP53) and the imprinted 11p15 region [11–13]. The gene discussed is TP53; the disease is Wilms tumor.