Over the past several decades, patients with mutations of the Wilms tumor suppressor-1 gene (WT1) have been categorized into three major subtypes: Denys–Drash syndrome (DDS), Frasier syndrome, and WAGR (Wilms tumors, aniridia, genitourinary anomalies, retardation) syndrome, primarily based on the phenotype (1–3). The gene discussed is WT1; the disease is Wilms tumor.