We report a case of cecal volvulus in an 11-year-old girl who had been diagnosed with Cornelia de Lange syndrome. She had undergone operative fundoplication several years earlier and was referred to our institute for treatment of intestinal obstruction. A severely dilated colon was detected on abdominal roentgenogram, and abdominal CT and colonic enema strongly suggested cecal volvulus. Emergency operation was performed and cecal volvulus with mobile cecum was ascertained; the volvulus was corrected and the mobile cecum was fixed to the peritoneum on the right side. Neurologically handicapped patients sometimes suffer from cecal volvulus, and patients with Cornelia de Lange syndrome are at especially high risk. Moreover, operative fundoplication promotes intestinal dilatation and it may be another risk factor for cecal volvulus.