Congenital diaphragmatic hernia (CDH) is a life-threatening disease in which the contents of the abdomen encroach upon the heart and lungs of the developing fetus. Despite many advances in managing the condition, it still persists as a major cause of neonatal death in newborns with severe respiratory failure. This review provides a brief context of CDH including its impact, clinical manifestations, and current management & limitations. A possible molecular treatment paradigm is also discussed based on studies linking retinoid signaling to CDH development. This evidence will outline the involvement of retinoid signaling pathways in lung & diaphragm development, the disruption of retinoid signaling resulting in CDH development, and a potential therapeutic modality by the manipulation of the retinoid signaling pathway.