Among the different involved genes15, WT1 germline mutations give rise to isolated WT, isolated forms of FSGS, and to two rare syndromes characterized by the association of male pseudohermaphrodism in 46,XY karyotype with FSGS and gonadoblastoma (Frasier syndrome), or with diffuse mesangial sclerosis and WT (Denys-Drash syndrome), reflecting the complex role of WT1 in cancer and embryonic urogenital development16. Here, WT1 is linked to Frasier syndrome.