Diaphragmatic eventration is an abnormal elevation of an intact diaphragm into the thoracic cavity as a result of paralysis, aplasia or atrophy of muscular fibers, which accounts for 5~10% of all diaphragmatic disorders. Congenital eventration result from a incomplete muscularization of the pleuroperitoneal membranes at 8~10 weeks' menstrual age, the cause of this failure is not known. Although some patients are asymptomatic and find out incidentally, significant compression of the affected chest contents can result in severe respiratory distress. The differentiation between congenital diaphragmatic eventration and congenital diaphragmatic hernia by sonography may be difficult, but important because of a significant differences in postnatal management and prognosis. We experienced a case of right congenital diaphragmatic eventration with severe respiratory distress during immediate postnatal period, who was initially diagnosed as congenital diaphragmatic hernia and performed plication at 18 hours after birth. So we report this case with review of literatures.