The authors present a clinical case of an extremely rare combination of the congenital defect of the front abdominal wall as omphalocele, congenital evolution defect of the small intestine - atresia of the small intestine type III, agenesis of the ileum with ileocecal angle and malrotation syndrome. Surgical treatment was as follows: T-shaped ileocolic «end-to-side» anastomosis with an unloading colostomy by the Bishop-Koop technique and intestinal intubaton till the Treitz ligament. Outcomes of such surgical correction are good.
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