Abstract
INTRODUCTION: This case report presents a rare occurrence of Type III Congenital Paraesophageal Hiatal Hernia (CPEHH) with Infantile Hypertrophic Pyloric Stenosis (IHPS) in a 28-day-old neonate. However, this unusual combination poses significant diagnostic and surgical challenges. PRESENTATION OF CASE: A 28-day-old male presented with respiratory distress and persistent vomiting. Imaging revealed CPEHH in the stomach and gastroesophageal junction in the thoracic cavity. IHPS was also identified during surgery. The patient underwent successful reduction of the hernia, pyloromyotomy, or Dor fundoplication with an uneventful recovery. DISCUSSION: The combination of CPEHH and IHPS is extremely rare, leading to diagnostic delays and increased risk of complications. Early surgical intervention is crucial in preventing severe outcomes such as gastric volvulus. CONCLUSION: This case underscores the importance of early diagnosis and coordinated surgical management in complex neonatal conditions, such as concurrent CPEHH and IHPS, contributing to the limited literature on this rare combination.