Objective To investigate the death risk factors of congenital diaphragmatic hernia (CDH). Methods The clinical data of children with CDH admitted from January 2017 to February 2021 were collected to analyze the risk factors for death of children with CDH. Results A total of 68 children (42 boys and 26 girls) with CDH were included. The median gestational age was 39.0 (37.5~39.4) weeks, the average birth weight was (2996.9±549.8) g, the PICU hospital stay was 19.2 (5.3-28.0) days and the invasive ventilation duration was 9.1 (3.9-13.9) days. There were 23 cases (33.8%) in the death group and 45 cases in the survival group. Fifty-nine cases underwent surgery, and 14 cases died after operation (23.7%). There were 54 cases of left diaphragmatic hernia and 14 cases of right diaphragmatic hernia. Compared with the survival group, the death group had smaller gestational age, lower birth weight, shorter PICU hospitalization and invasive mechanical ventilation time, lower observed/expected lung to head ratio, lower pH value, higher proportion of low Apgar scores (1-minute Apgar score ≤7) and liver herniation, higher pulmonary artery pressure and oxygenation index (OI), and the differences were statistically significant (P<0.05). Binary logistic regression analysis showed that OI and hepatic herniation were independent risk factors for death of CDH children (P<0.05). Conclusions CDH has a higher mortality and the risk of death increases with the increase of OI. Children with liver herniation had increased risk of death.