Malrotation of the intestinal tract is a product of a well defined aberrant embryology. Because the consequences of malrotation associated with a midgut volvulus may be catastrophic, an understanding of the anatomy, diagnostic criteria, and appropriate therapy for this putative emergency illness is imperative. This report summarizes a recent 18-month experience with this diagnosis and contrasts this experience with that in the published literature. More than half (14/22) of the patients presented during the first month of life, and all had vomiting, which in most cases was bilious. The barium upper gastrointestinal series was the preferred diagnostic study, being both sensitive (18/19, 95%) and accurate (18/21, 86%). In this series two-thirds of the patients presented with volvulus (15/22, 68%) of whom five had ischemic intestine requiring resection. One of these children died of overwhelming sepsis. A Ladd procedure was the preferred treatment, which as defined by us includes evisceration and inspection of the mesenteric root, counterclockwise derotation of a midgut volvulus, lysis of Ladd's bands with straightening of the duodenum along the right abdominal gutter, inversion-ligation appendectomy, and placement of the cecum into the left lower quadrant. A high index of suspicion in the neonate with vomiting, rapid diagnosis, and appropriate operative therapy results in a predictable favorable outcome for children with intestinal malrotation.