Abstract: Objective: To explore the clinical features, surgical treatment and prognosis of neonates with diaphragmatic eventration. Methods: Twenty-two neonates with diaphragmatic eventration admitted from June 1st, 2016 to May 31st, 2020 were recruited in the study. Clinical data was analyzed including gender, gestational age, weight, sides, being congenital or acquired, degree of diaphragmatic eventration, combined congenital diseases, treatment, preoperative/postoperative respiratory support, prognosis, et al. Clinical characteristics of surgical cases and survived cases without surgery were compared as well. Results: The median gestational age and birth weight of 22 neonates, including 12 males and 8 preterm infants, were 38 (33, 39) weeks and 2,730 (1,940, 3,110) g respectively. All cases were unilateral, with 20 cases on the right side. Twenty cases were congenital while 2 cases were secondary to birth injury. The median elevation of diaphragmatic eventration was 1.75 (1.50, 2.00) cm. Nine cases had operation. The lowest weight of operated cases was 2.3 kg at the time of operation. The main indications for surgery were failure to maintain normal breathing (8 cases), and repeated cyanosis (1 case). Compared with non-surgical survival, the diaphragm elevation ［2.0 (1.65, 2.4) vs 1.60 (1.0, 1.80) cm, P=0.015］, and the proportion of respiratory symptoms as the chief complaint for admission (100% vs 50%, P=0.029) were higher in surgical cases. Co-existing congenital diseases in recruited cases were common, including congenital hypotonia, congenital heart disease, airway anomalies, cryptorchidism, et al. All 5 deaths were combined with other congenital abnormalities, including 4 cases of congenital hypotonia and 1 case of complicated congenital heart disease. Conclusion: The main indication for operation in neonatal diaphragmatic eventration cases was failure to maintain normal breathing. The poor prognosis was mainly determined by combined critical congenital anomalies, such as congenital neuromuscular disease.