Midgut volvulus secondary to intestinal malrotation usually presents within the first month of life. Diagnostic delay may result in midgut infarction and mortality. In this retrospective study, we review seventeen cases of midgut volvulus to assess the importance of early recognition for midgut volvulus in pediatric patients of any age.. These patients were diagnosed as having a midgut volvulus by operation at Ewha Womans University Hospital. Eleven patients (64.7 %) were less than 1 month of age, and fifteen were boys (88.2 %). The mean gestational age was 38.3 weeks and the birth weight was 3.1 kg. Eight patients (47.1 %) had one or more combined anomalies such as heart malformation, brain ischemia, Down's syndrome or duodenal atresia. Vomiting was the most common symptom. Only thirteen patients underwent preoperative diagnostic procedures; 13 abdominal sonography demonstrated the whirlpool sign in 8 patients, upper gastrointestinal tract roentgenography showed a cork-screw pattern in 7 patients, and barium enema or small bowel series demonstrated positive findings in 7 patients. A Ladd's procedure was was formed on all patients.. There was no mortality or severe morbidity such as short bowel syndrome. Midgut volvulus should be included in the differential diagnosis in any infant or child who presents with the symptoms of acute abdomen, especially with vomiting.