An analysis of ClinVar (https://www.ncbi.nlm.nih.gov/clinvar) for pathogenic variants involving the ZF4 of WT1 indicated several missense variants (p.Pro487Ser, Variation ID: 543123; p.Ser488Asn [x2], Variation ID: 578607; p.Lys491Arg, Variation ID: 655847; p.His505Gln, Variation ID: 664113) in association with the typical clinical features of WT1 pathogenic variants (Denys–Drash syndrome, Wilms tumor, aniridia, genitourinary anomalies, mental retardation syndrome, Frasier syndrome). This evidence concerns the gene WT1 and Frasier syndrome.