Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that allows abdominal viscera to herniate into thoracic cavity. Despite of the improvements in the intensive neonatal care results of the treatment of this condition remain rather poor with an overall survival rate estimated at 50%. These unsatisfactory results stem from the complications which appear in the early neonatal period. The most difficult outcome of herniation is development of the pulmonary hypoplasia and an increased risk of persistent pulmonary hypertension of newborn (PPHN). This is seen already in the first day of life as newborn shows signs of respiratory distress syndrome. Should CDH be suspected, a screening with pulse oximetry should be conducted and final diagnosis based on findings of echocardiography. Current treatment of the persistent pulmonary hypertension of newborn includes general supportive therapy of cardiorespiratory system with volume, inotropic agents and oxygen as well as treatment of the underlying acidosis. When needed, treatment can also involve supplemental therapy such as vasodilator agents and extracorporeal membrane oxygenation (ECMO). New treatment protocol focuses on preoperative stabilization of pulmonary hypertension and lung protective mechanical ventilation using small volumes, high frequency and low peak inspiratory pressure. Despite high expectations of ECMO support, it has recently been showed that ECMO is of critical importance only in severe cases for which conventional measures remain insufficient. An operation of diaphragmatic defect follows after clinical stabilization. Recurrence of the pulmonary hypertension presents the most difficult postoperative complication. Other postoperative complications include chylothorax, which is best treated with reduction of lymph production, drainage and obliteration of pleural space. In addition, the patient should be supported with fluid, electrolytes and nutrients and respiratory stabilized.