[6] We report here the case of a 46, XX female infant who presented with HUS and biopsy-proven thrombotic microangiopathy (TMA) in the setting of a heterozygous mutation in the Wilms tumor 1 (WT1) gene that is the most common cause of Denys-Drash syndrome (DDS), which includes a pathognomonic glomerulopathy, diffuse mesangial sclerosis. This evidence concerns the gene WT1 and Diffuse mesangial sclerosis.