The purpose of this study was to demonstrate survival rate changes after the introduction of inhaled nitric oxide (iNO) therapy, and to identify the factors that influence these changes in neonates with a congenital diaphragmatic hernia (CDH) at a single center. A total of 48 neonates were divided into two groups based on the time of admission, i.e., into period I (P1; n=17; before the introduction of iNO therapy) and period II (P2; n=31; after the introduction of iNO therapy). Survival rates of the 48 neonates showed a tendency to increase from 53% during P1 to 77% during P2, but without a statistical significance, but a significant difference was found between survival rates during the two periods after adjusting for initial clinical characteristics, when the postoperative survival rate increased significantly from 69% for P1 to 100% for P2. The mean duration of preoperative respiratory management was significantly longer for P2 than for P1. Seven of 12 patients who received preoperative iNO therapy due to persistent pulmonary hypertension or refractory preductal hypoxemia in P2 survived after operation. We speculate that a management strategy based on iNO therapy and delayed operation, rather than differences between the initial clinical characteristics of the two study groups, might partially contribute to the observed improvements in postoperative and overall survival rates in neonates with CDH.