Background. Late-presenting congenital diaphragmatic hernia occurs beyond the neonatal period, and is relatively rare, presenting with nonspecific respiratory and gastrointestinal symptoms.

Case. We report a rare case of late-presenting congenital diaphragmatic hernia in a 7-year-old girl, who presented with abdominal pain, shortness of breath and fever on admission. Work-up revealed intrathoracic gastric perforation, acute pancreatitis and septic shock with a diaphragmatic defect. Due to the high content of amylase in pleural effusion, we suspected the presence of a pancreaticopleural fistula, and we were also puzzled whether the gastric perforation was caused by a pleural indwelling catheterization, but this was ruled out. We about performed a laparotomy to reposition the herniated organs, repair the hernia and the gastric perforation, and undergo the gastrostomy. The girl had an uneventful post-operative recovery.

Conclusions. Late-presenting congenital diaphragmatic hernias are often misdiagnosed. Clinicians should combine multiple imaging modalities to make a definite diagnosis and perform surgery as soon as possible to avoid severe complications.

Keywords: acute pancreatitis, children, gastric perforation, hydropneumothorax, late-presenting congenital diaphragmatic hernia