Two children had a pathogenic variant in WT1 and 1 each in NPHS2, PLCE1, COL4A3, LAMB2 and NPHP1. One child with WT1 had clinical phenotype of Frasier vs Denys Drash syndrome (Case 37) and other with WT1 (Case 67) had SRNS without any other syndromic feature. The gene discussed is COL4A3; the disease is Denys-Drash syndrome.