Disease-causing variants in the zinc finger DNA-binding protein WT1 are associated with five related autosomal dominant disorders: type 1 Wilms’ tumor (OMIM 194070), Denys–Drash syndrome (OMIM 194080), Frasier syndrome (OMIM 136680), Meacham syndrome (OMIM 608978), and nephrotic syndrome type 4 (NPHS4; OMIM 256370). The gene discussed is WT1; the disease is familial idiopathic steroid-resistant nephrotic syndrome.