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This study aims to assess the procedure-related complications and survival following fetoscopic endotracheal occlusion (FETO) for severe congenital diaphragmatic hernia.A systematic review and meta-analysis of PubMed and Scopus database searching for FETO procedure in severe CDH (lung-to-head ratio [LHR] < 1.0 and/or observed/expected LHR < 0.26 and > 1/3 liver herniation) were performed. The relative risk was assessed and 95% confidence interval (CI) calculated. Procedure complications and survival were compared between FETO and randomized controlled trial (RCT) as well as observational case-control studies.A total of 4,807 records were retrieved based on the title and abstracts, and 18 studies were eligible for statistical analysis (1 RCT and 17 observational case-control studies). Relative risk (95% CI) comparing FETO and controls for premature rupture of membrane, preterm birth < 32 weeks, preterm birth < 37 weeks, survival at 30 days, and survival at 6 months were 1.7 (0.8-2.4), 7.3 (0.4-134), 1.8 (0.8-3.9), 5.8 (1.5-22.9), and 10.5 (1.5-74.7), respectively. Mean difference (95% CI) for gestational age at delivery comparing FETO and controls was 1.8 (−3.1 to −0.5). All these outcomes showed a low level of evidence.FETO procedure increased the neonatal survival at 30 days and 6 months; however, it presented a higher rate of premature rupture of membrane, preterm birth < 37 weeks, and decreased the gestational age at delivery by 2 weeks. Nonetheless, the level of evidence is low for all these outcomes. We suggested a large international multicenter RCT to prove the real benefits of FETO.