Intestinal malrotation is usually asymptomatic and most often is found during abdominal exploration for other surgical diseases. However, a serious complication of intestinal malrotation, midgut volvulus is a true surgical emergency of childhood. The clinical findings, diagnostic procedures, treatment, and prognosis of midgut volvulus were reviewed by a retrospective study. Between 1980 and 2005, 29 patients with midgut volvulus ranging in age from 1 day and 15 years were treated at HanYang University Hospital. Seventy-nine percent of the patients presented before 1 month of age. Midgut volvulus occurred 2 times more frequently in male. The clinical findings were bilious vomiting (96.6 %), irritability (34.5 %), abdominal distention (13.8 %), abdominal pain (10.3 %), and palpable abdominal mass (6.9 %). The diagnosis was made by abdominal simple x-ray (17.2 %), upper gastro-intestinal contrast study (37.9 %), abdominal sonogram (20.7 %), abdominal CT (3.4 %), and abdominal exploration (20.7 %). Among the 29 patients, 5 patients developed gangrene of small intestine due to strangulation and underwent resection of bowel. Two patients died due to sepsis.