Midgut malrotation with volvulus is a life-threatening surgical emergency. Because the consequences of malrotation associated with midgut volvulus may be catastrophic, prompt diagnosis and treatment are required to prevent mortality and short-gut syndrome. Intestinal malrotation is usually observed in the neonatal period. Bilious vomiting and bloody stools are the two most common clinical presentations, but the patients exhibit often no abnormal physical findings on abdominal examination. Imaging techniques (Rx, US) of the upper gastrointestinal tract must be very accurate in order to diagnose volvulus but frequently they fail to identify this illness. If any doubt exists, refer with pediatric surgical support for consultation.