This manuscript is dedicated to Baby Lucy and her parents for the contribution that they have made to helping us to understand developmental diaphragmatic and lung defects. This work was funded by National Institute of Child Health and Human Development (NIHCD) grant HD36404 (DRB) and the Hearst Foundation (KGA). KGA also received salary support from NICHD grant T32HD040129–02. We would like to thank Stuart Orkin, M. D., for the generous gift of his Fog2 −/− mice. We would also like to thank Raju Kucherlapati, Ph. D., and Birgit Funke, Ph. D., for providing control DNA samples. Competing interests. The authors have declared that no competing interests exist. Author contributions. KGA, JJG, and DRB conceived and designed the experiments. KGA, BJH, HH, SGT, LK, CR, RPB, and JAE performed the experiments. KGA, BJH, HH, LK, CR, JAE, JJG, and DRB analyzed the data. KGA, SOV, SGT, BRP, JAE, JJG, and DRB contributed reagents/materials/analysis tools. KGA and DRB wrote the paper. Abbreviations CDH congenital diaphragmatic hernia E[number] embryonic day [number] ENU N-ethyl-N-nitrosourea HGF Hepatocyte growth factor/Scatter factor lil little lung PPF pleuroperitoneal fold