Abstract
Congenital diaphragmatic hernia (CDH) is encountered in just under 1 in 6000 live births, while congenital duodenal obstruction is seen once every 8000 live births. These congenital anomalies have only been reported together as part of the VACTERL syndrome and therefore in isolation represent an incredibly rare occurrence. This is a case report of a girl born at 34 weeks gestation who had an antenatal diagnosis of left CDH. Five days following operative repair of this, there was extensive pneumoperitoneum and pneumothorax. Upper gastrointestinal contrast study showed a perforation of the duodenum and at laparotomy, a duodenal web was found in the fourth part of the duodenum with perforation immediately proximal. Following duodenoduodenostomy, the postoperative recovery was good. She achieved full enteral feeds and was discharged home. This case highlights the importance of considering rare associations if postoperative recovery is not as expected to prevent delay in undergoing definitive treatment.