Congenital abdominal wall defects are omphalocele, gastroschisis and umblical cord hernia (UCH) in order of frequency. Etiologies and the morphological appearance of these abnormalities are different. UCH is a rare abnormality of the anterior abdominal wall and, often miscategorized as omphalocele minor. Although the UCH cases with a variety of intestinal diseases reported in the literature the case with ileocecal junction atresia hasn't been found. In this study, a case with UCH is presented caused by ileal dilatation due to ileocecal junction atresia. Copyright © 2012 by Türkiye Klinikleri.