This report concerns 447 infants and children with anomalies of rotation and fixation. Patients were placed in four groups based on initial symptoms. Group A involved 18 patients with acute midgut volvulus. At laparotomy, midgut volvulus was noted and reduction of midgut volvulus and a Ladd procedure were performed in 10 cases and resection was required in 8. There were five deaths (28%). Group B included 54 children with chronic symptoms of intermittent volvulus or duodenal obstruction. Group C involved 44 cases of malrotation observed during exploration for other disorders. Patients in groups B and C underwent a Ladd procedure and appendectomy. There were five unrelated deaths. Group D included 331 neonates with malrotation caused by either diaphragmatic hernia (n = 111) or abdominal wall defects (n = 220). A Ladd procedure was performed on 48 patients with abdominal wall defects and 29 surviving children with diaphragmatic hernia. Only 2 of 172 (1.2%) patients with abdominal wall defects and 1 of 34 (2.9%) patients with diaphragmatic hernia not treated for malrotation had midgut volvulus. Midgut volvulus is more common in infants and is associated with a high mortality rate (28%). Patients with malrotation and chronic obstructive symptoms or those observed during other elective procedures should undergo a Ladd procedure because of the risk of midgut volvulus. The risk of midgut volvulus is low in patients with abdominal wall defects and, probably as a result of adhesions from previous neonatal operations.