Acute gastric volvulus associated with congenital diaphragmatic hernia is an unusual surgical emergency. We describe a case of an 11-year-old girl who presented with a 4-day history of abdominal pain, nonproductive retching, cough, and shortness of breath. A chest radiograph revealed a large air-fluid level in left hemithorax and the presence of intestinal loops with marked mediastinal deviation. Nasogastric decompression was unsuccessful. Via a thoracoscopic approach, the large fluid-filled stomach was percutaneously decompressed but could not be reduced. Through a left subcostal incision, a left-sided diaphragmatic defect about 4 x 5 cm was encountered. A large portion of small intestines, ascending and transverse colon, strangulated but viable stomach, and a large spleen herniated through the defect. The contents were reduced, revealing a combined gastric volvulus. Once the diaphragmatic defect was repaired primarily, there was insufficient space in the abdominal cavity to contain all the viscera reduced form the chest. Therefore, we placed an AlloDerm patch on the fascia and closed with a wound V.A.C (Kinetic Concepts Inc, San Antonio, TX). Two weeks later, the wound was definitively closed; she recovered uneventfully and was discharged home 3 days later. To our knowledge, only 26 previous cases of acute gastric volvulus complicating a congenital diaphragmatic hernia in children have been reported in the literature. Our patient represents the 27th case and the first combined type acute gastric volvulus case.