This is a review of 12 cases with apple peel small bowel with agenesis of the dorsal mesentery and intestinal atresia, 11 of them jejunal and 1 duodenal in type who were treated at the Clinica Infantil "La Paz" Madrid over a period of ten years (1969--1979). The associated anomalies, the surgical treatment and the cause of death as well as the etiology and pathogenesis of this malformation are discussed.