Atresias of the intestinal tract are one of the causes of congenital intestinal obstruction. The superior mesenteric artery supplies the midgut from the fourth portion of the duodenum to the midportion of the transverse colon. Absence of the superior mesenteric artery (SMA) with subsequent absence of jejunum, ileum, appendix, and right colon is a previously unreported condition. A fetal vascular accident might result in this intestinal catastrophe.