Jejunoileal atresia, defined as a congenital defect in continuity of the small bowel, is a common cause of intestinal obstruction in the newborn.1 3 The incidence of jejunoileal atresia varies between 1 in 1500 and 1 in 3000 live births.4 Jejunoileal occlusions occur more frequently than duodenal or colonic atresias.1 , 5 With improved neonatal and perioperative care, safe anesthesia, refined surgical techniques, and management of short bowel syndrome, a survival rate of greater than 90% can be expected in well-resourced centers. At the Red Cross War Memorial Children’s Hospital in Cape Town during the 56 years from 1959 to 2015, 363 jejunoileal atresias, 275 (76%) jejunum and 88 (24%) ileum, were seen (Table 58.1). The mortality rate was initially high, and it was only in the mid-1950s that an improved understanding of the pathogenesis and pathology of the condition led to innovative surgical techniques, which resulted in greatly improved surgical outcome.4 6