Background: Congenital obstruction of the gastrointestinal tract in the form of complete atresia occurs in newborns in 60% of all cases of obstruction of the digestive tract, while agenesis of the derivative of the midgut is a rather rare defect. Case presentation: After birth, the child in this case was repeatedly operated on for congenital intestinal obstruction. Most of the jejunum, ileum, cecum, ascending, right half of the transverse colon were absent in the child, and an anastomosis was performed between the jejunum and transversum. In the postoperative period, it was not possible to cope with the intestinal and protein -energy deficiency and the child exerted at home. The section has confirmed clinical diagnoses. Conclusion: The authors draw attention to the possibility of resorption of the extraperitoneally located intestine at the end of the first period of intrauterine rotation with a possible vascular and/or ischemic disaster.