<h4>Background</h4>Denys-Drash syndrome (DDS) is a rare genetic disorder characterized by mutations in the Wilms tumor suppressor gene (<i>WT1</i>), leading to a triad of conditions including nephrotic syndrome progressing to end-stage renal disease (ESRD), Wilms tumor, and ambiguous genitalia. The gene discussed is WT1; the disease is Wilms tumor.