**1. Introduction**

CDH is a rare and major congenital anomaly. It is characterized by the partial or complete absence of diaphragm on one or both sides with herniation of abdominal content into the thorax. The dual-hit hypothesis, proposed by Keijzer et al, has suggested that the early insult in lungs' development is bilateral, occurring before and independent of the diaphragmatic defect; and a later second hit to the ipsilateral lung via compression from the herniated abdominal content leads to the characteristic pulmonary hypertension and hypoxemia in the neonate [1].

Advanced prenatal evaluation and a multidisciplinary perinatal management approach have contributed significantly to the improvement in the outcome of CDH. Several prognostic indicators have been forwarded in an attempt to identify candidates with better outcome potential who could benefit maximally from the antenatal and postnatal interventions. CDH is considered to be a medical emergency and the initial intensive management is determined by the severity of the cardiorespiratory failure, which is a consequence of lungs hypoplasia, pulmonary

vascular maldevelopment, and the ventricular dysfunction, the three outstanding pathological features of the anomaly. The surgical repair is generally deferred by consensus among the neonatologists and the surgeons. The management is more complicated if CDH is associated with other organ anomalies, which might make the outcome worse. CDH is one of the most challenging morbidities in the neonates. In this article, an evidence based overview of the current status of the disease entity is provided.
