OBJECTIVE: Hiatus hernia is frequent in adults and rare in children; congenital hiatus hernia is even rarer. In this study, wedescribe a group of infants with congenital hiatus hernia and discuss its management.METHODS: Records of patients (male: 3, female: 4) who were diagnosed with congenital hiatus hernia between 2010 and2016 were extracted. Demographic data, presenting symptoms, diagnostic investigations, operative details, postoperativefollow-up, and early and late postoperative complications were evaluated retrospectively.RESULTS: Four patients were female and three were male. One patient was diagnosed prenatally while the mean age atdiagnosis for others was 18.6 months. Four patients had type IV hernia, 2 had type III hernia, and one had type I hernia. Thediagnosis was confirmed by chest X-ray, computerized tomography (CT) and/or upper gastrointestinal series. The hiatal repairwas done in all patients either by laparotomy or laparoscopy. During the procedure, 2 patients had Nissen fundoplication and3 patients had Thal fundoplication. Recurrence of hernia occurred in the 2 patients who had Thal fundoplication.CONCLUSION: Recurrence of sliding hernias with Thal fundoplication seem more frequent in the series. If the esophagogastric junction is present in the thorax, mediastinal dissection of the esophagus may be required to achieve a good abdominalesophagus structure, which will prevent a recurrence.