Denys Drash Syndrome - A rare cause of SRNS, Male Pseudo-Hermaphroditism with Wilms Tumor

Back ground - Denys Drash syndrome is a very rare disorder characterized by gonadal dysgenesis, nephropathy and wilms tumor. Case report - A 1 year 6 months boy presented with bilateral undescended testes, mid penile hypospadias and abdominal mass. On evaluation found to have wilms tumor, for which left nephrectomy was done. He was treated with chemo radiotherapy postoperatively. Orchidopexy of both testes was done in stages following which he developed nephrotic syndrome-resistant to steroids. Thus Denys Drash syndrome was made out. He is on Mendoza regimen currently for SRNS. He has completed chemotherapy and is on regular follow up for Wilms tumor and SRNS and is now 3 years.