The association between CDH and CNS is uncommon, with only a few reported cases, including a case of idiopathic nephrotic syndrome combined with esophageal diaphragmatic hernia reported by Galloway and Mowat in 1968 [4], and a report of nephrotic syndrome with WT1 mutations, in which diaphragmatic defects or hernias were rarely associated with each other [1]. Here, WT1 is linked to nephrotic syndrome.