Despite major insights into the pathogenesis and pathophysiology of congenital diaphragmatic hernia, and despite the availability of an antenatal diagnosis and continuous progress in neonatal intensive care, little improvement has been obtained in the prognosis of this malformation. Thus obstetricians, neonatologists and pediatric surgeons are still facing a several dilemma: dilemma before birth to predict the prognosis, i.e., to evaluate the severity of the associated pulmonary hypoplasia in order to decide whether or not to interrupt pregnancy; dilemma after birth in case of severe respiratory failure to decide how far to go in life support. Based on a review of the literature and their own experience, the authors attempt to recapitulate the perinatal management and outcome of this severe malformation.