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In the article, the authors present the case of a girl with multiple congenital malformations of the abdominal organs, one of them being duodenal obstruction and the other non-rotation of the intestine, with typical clinical symptoms and signs of duodenal obstruction and non-rotation. The importance of diagnostic imaging and co-operation between the pediatric gastroenterologist, radiologist and abdominal surgeon is emphasized. The authors caution that this condition can be associated with high mortality and morbidity rates when it is not recognized early and treated correctly.