Childhood interstitial lung disease (chILD) is on the rise as an indication for pediatric LTX, at least in the US.11, 20 These are heterogeneous disorders, with diagnostic criteria established by respiratory and chILD societies,8, 29 which include but are not limited to surfactant protein (SP) disorders, lung growth abnormalities (including alveolar capillary dysplasia with misalignment of pulmonary veins), pulmonary fibrosis, interstitial pneumonias, and entities being increasingly recognized like chILD associated with STAT3 or COPA (coatomer protein complex subunit alpha) mutations. Here, COPA is linked to alveolar capillary dysplasia with misalignment of pulmonary veins.