The aim of this study was to analyze pitfalls in the diagnosis and treatment of congenital paraesophageal hiatal hernia (PEHH).Methods: Between 1992 and 2004, the records of 5 infants with PEHH were retrospectively reviewed for age, sex, presenting symptoms, radiological studies, operative findings and approaches, and outcomes. Results: All cases (3 male, 2 female) had right-sided hernias. They had clinical features of recurrent chest infections and intermittent vomiting that were present since birth in 3. Three presented acutely ill with findings of respiratory distress and vomiting. Three were referred with misdiagnoses of reflux disease, thoracic mass, and bronchopneumonia. On the chest x-rays of 3 cases, there were paracardiac opacities suggesting a mass lesion. According to the upper gastrointestinal series and/or computed tomography findings, 4 cases had a combination of sliding and paraesophageal hernia, and the remainder one had pure rolling hiatus hernia. Three had obstruction owing to organoaxial volvulus and required an emergency operation. All cases had a large hernia orifice. Four had gastroesophageal junction (GEJ) displaced into the thorax, and in 3, the stomach was found to be twisted, and transverse colon with omentum was also in the thorax in 2. In the remainder, the GEJ was in its normal position with herniated stomach. None of the cases had normal gastrosplenic and gastrocolic ligaments. Surgical repair included resection of the sac, closure of the hiatal defect, and Thal procedure. Two had intestinal malrotation, with right ovarian torsion and ventricular septal defect, respectively. Postoperative ventilation was required in one who later died. At a mean follow-up of 2 years, the other 4 had no symptoms related to the disease, and no evidence of recurrence or reflux was noted on control upper gastrointestinal series. Conclusion: Congenital PEHH may be difficult to diagnose. It is frequently complicated and associated with morbidity and even mortality. If the defect is large and associated with displacement of GEJ into the thorax, adding an antireflux procedure to the repair is appropriate. The aim of this study was to analyze pitfalls in the diagnosis and treatment of congenital paraesophageal hiatal hernia (PEHH). Methods: Between 1992 and 2004, the records of 5 infants with PEHH were retrospectively reviewed for age, sex, presenting symptoms, radiological studies They had clinical features of recurrent chest infections and intermittent vomiting that were present since birth 3. The three cases of acutely ill with findings of respiratory distress and vomiting. Three were referred with misdiagnoses of reflux disease, thoracic mass, and bronchopneumonia. On the chest x-rays of 3 cases, there were paracardiac opacities suggesting a mass lesion. According to the upper gastrointestinal series and / or computed tomography findings, 4 cases had a combination of sliding and paraesophageal hernia, and the remainder one had pure rolling hiatus hernia. Three had obstruction Both had a large hernia orifice. Four had gastroesophageal junction (GEJ) displaced into the thorax, and in 3, the stomach was found to be twisted, and transverse colon with omentum was also in the thorax in 2. In the remainder, the GEJ was in its normal position with herniated stomach. None of the cases had normal gastrosplenic and gastrocolic ligaments. Surgical repair included the resection of the sac, closure of the hiatal defect, and Thal procedure. Two had intestinal malrotation, with right ovarian torsion and ventricular septal defect, respectively. At a mean follow-up of 2 years, the other 4 had no symptoms related to the disease, and no evidence of recurrence or reflux was noted on control upper gastrointestinal series. Conclusion: Congenital PEHH may be difficult to diagnose. It is frequently complicated and associated with morbidity and even mortality. If the defect is large and associated with displacement of GEJ into the thorax, adding an antireflux procedure to the repair is appropriate.