In summary, our results demonstrate that the congenital deletion of Nedd4-2 in lung epithelial cells causes severe ILD in neonatal mice that shares key features with interstitial lung diseases in children (chILD), including respiratory distress, hypoxemia, growth failure, sterile alveolitis, progressive fibrotic remodeling of the lung parenchyma and high mortality. Here, NEDD4L is linked to hypersensitivity pneumonitis.