A case of acute abdomen due to diastasic perforation of the right colon by volvulus on mesenterium commune with associated agenesis of the left kidney and urogenital malformations in a young woman is reported. The case emphasizes the complexity and the polymorphism of congenital abdominal malformations, particularly predisposing to volvulus, that can represent a really challenge for emergency surgeon. The A.A. analyze literature about congenital intestinal anomalies to evaluate clinical, diagnostic and therapeutical problems, and they remark features to take into consideration in emergency: clinical history in non-operated patient of abdominal pain and biliary vomit with spontaneous resolutions; nutritional compromise; hypothetical diagnosis of anorexia or food intolerance; malformations in other organs or systems and, one mostly, young age. The correct interpretation of these symptoms can suggest suspicious of an occlusive complication due to intestinal malformations. Radiological data and the exploratory laparotomy or laparoscopy will confirm our intuition. If otherwise diagnosis of the cause underlying the chronic recurrent abdominal pain are delayed, surgical operation in emergency condition will be more aggressive and anatomical structures sacrificed.