Germline missense mutations in WT1 exon 8 or 9 are associated to Denys-Drash syndrome (pseudo- or true hermaphroditism, nephropathy, and genital anomalies with an estimated risk for WT higher than 90%) [8] while germline, point mutations in the WT1 intron 9 donor splice site account for several cases of WT associated to Frasier syndrome (pseudohermaphroditism and progressive glomerulopathy) [9, 10]. Here, WT1 is linked to Denys-Drash syndrome.