A newborn boy with aplasia cutis congenita had biliary atresia, distal duodenal atresia, and a severe infarct of the intestine resulting in complete absence of the entire midgut. The boy died due to biliary atresia and severe short gut syndrome approximately 3 weeks after birth. While the association of duodenal atresia with aplasia cutis congenita has been described, the findings of biliary atresia and midgut atresia in association with aplasia cutis congenita have not been described previously.