Familial Jejunal Atresia with ‘Apple-Peel’ Variant 1

We report two siblings who had jejunal atresia which we believe to be familial. The parents of these siblings were first cousins. The first child had jejunal atresia with mesenteric agenesis and ‘apple-peel’ configuration; the second child had jejunal atresia with a V-shaped mesenteric defect. Other reported cases of familial atresia of the small intestine are reviewed.