A 20-year-old woman with glycogen storage disease type 0 (GSD-0) underwent velopharyngeal closure for velopharyngeal insufficiency following palatoplasty. To reduce the risk of complications attributed to GSD-0, general anesthesia was administered using a total intravenous anesthesia (TIVA) technique with propofol and remifentanil, along with supplemental glucose-containing intravenous fluids. Her blood glucose remained stable, intraoperative body temperature ranged from 36.5 to 37.2°C, and the velopharyngeal closure was completed without any adverse events.