WT1-related nephropathy can occur in isolation, or as part of urogenital malformation syndromes and disorders of sex development, including Wilms tumor, aniridia, genitourinary anomalies, mental retardation syndrome (WAGR, OMIM 194,072); Frasier syndrome (FS, OMIM 136,680); Denys-Drash syndrome (DDS, OMIM 194,080); and, rarely, Meacham syndrome (MS, OMIM 608,978), the latter 3 being allelic disorders with an overlap of clinical features [1]. The gene discussed is WT1; the disease is Wilms tumor.