This study demonstrates that the congenital deletion of Nedd4-2 in lung epithelial cells causes a spontaneous and rapidly progressive lung disease in neonatal mice that shares key clinical and histopathological features of interstitial lung diseases in children (chILD), and thereby extends recent reports on the E3 ubiquitin ligase NEDD4-2 in the pathogenesis of ILD [9]. Here, NEDD4L is linked to interstitial lung disease.