Intestinal malrotation is a congenital abnormal fixation of the bowel that predisposes to life threatening midgut volvulus. Most events of midgut volvulus occur in the first month of life with decreased risk with age. The most typical presentation is bilious vomiting. Upper gastrointestinal series is the study of choice for diagnosis of malrotation. For optimal results, the study should be well planned with meticulous attention to technique. In equivocal cases, small bowel follow-through study and ultrasound can be performed.