The association between umbilical cord ulceration and congenital intestinal atresia is being increasingly reported and carries a high mortality. We report on a case of jejunal atresia associated with massive fetal haemorrhage from an umbilical cord ulcer. Fetal distress noted on continuous fetal heart monitoring allowed for delivery by emergency caesarean section followed by appropriate neonatal resuscitation and intact survival. This and other reported cases highlight the need for identification of high-risk fetuses with congenital intestinal atresia. Close fetal monitoring during labour is imperative in order to improve outcomes.