Umbilical cord ulceration has been associated with congenital upper intestinal (duodenal or jejunal) atresia and can lead to fatal fetal intrauterine hemorrhage. We report a case of spontaneous hemorrhage from the umbilical cord, incidentally noted at the time of ultrasound in a 33-week fetus with suspected duodenal atresia, in which immediate delivery resulted in a good outcome. Despite many reports in the literature of congenital upper intestinal atresia and its association with umbilical cord ulceration, the propensity for this lesion for fetal hemorrhage, and the resulting perinatal morbidity and mortality, there appears to be a gap in the dissemination of this knowledge. In fetuses with suspected congenital upper intestinal atresia, recognition of the entity of umbilical cord ulceration may be improved by ultrasound with special attention to the amount of Wharton’s jelly within the cord. Routine antepartum fetal surveillance may reduce perinatal morbidity and mortality from this condition. A high index of suspicion is needed to make the diagnosis of umbilical cord ulceration in association with congenital upper intestinal atresia. The role of amniotic fluid bile acids in the genesis of this disorder needs further study.