In the recent years, the association between congenital intestinal atresia and umbilical cord ulceration has been demonstrated; however, this complication is rarely encountered. To the best of our knowledge, only 14 cases on this association have been published previously. We encountered a case of acute intrauterine hemorrhage from an umbilical cord ulcer in a fetus with duodenal atresia that resulted in early neonatal death. There is little information available on the association between congenital intestinal atresia and umbilical cord ulceration. Greater understanding may alter the obstetric management. Although the prevention of sudden perinatal death is considered to be difficult, frequent fetal monitoring and detailed ultrasonographic examinations may facilitate the early detection of this condition, thereby enabling the rescue of the affected fetuses. Further investigation is required to establish the management protocol.