Developmental syndromes such as WAGR (Wilms’ tumor, aniridia, genital anomalies and retardation) and DDS (Denys–Drash syndrome) are associated with WT1 pathogenic variations and were shown to have an increased risk of WT development (30–75%) and lower mean ages of WT diagnosis [8, 9]. The gene discussed is WT1; the disease is Nephroblastoma.