DDS, mostly caused by mutations in exon 8 or 9 of WT1 is characterized by congenital/infantile NS, ambiguous genitalia, and a high risk for Wilms tumor while FS caused by mutations in the donor splice site at intron 9 is characterized by SRNS due to focal segmental glomerulosclerosis (FSGS), gonoadoblastoma and 46 XY disorder in sex development with sex reversal [10–12]. The gene discussed is WT1; the disease is Denys-Drash syndrome.