The association between umbilical cord ulceration and intestinal atresia has been previously established. Umbilical cord ulceration is a seemingly rare, potentially life threatening complication of intestinal atresia. The exact etiological mechanism is unknown and recent literature indicates more intensive prenatal monitoring may alter fetal outcome. In this paper we will review the previously reported cases of intestinal atresia complicated by umbilical cord ulceration and comment on the pathophysiological mechanism of umbilical cord ulceration, with emphasis on the unique location of the atresia at the gastroduodenal junction in our case.