The management of neonates with high risk congenital diaphragmatic hernia (CDH) which gives rise to respiratory distress syndrome in 6 hours after born is so troublesome that the mortality is very high. The past advocation that CDH should accept emergency operation has been called in question. Another therapeutic procedure is recommended that CDH repair operation should be performed after the respiration and circulation has been stabilized. This procedure has presented better effect than before. The present advances in the management including general measures, mechanical ventilation, extracorporal membranous oxygenator (ECMO), nitric oxide (NO) inhalation, glucocorticoid, pulmonary surfactant, CDH repair and so on were reviewed.