Freeman-Sheldon syndrome is a rare congenital myopathy principally characterized by facial and skeletal abnormalities. We report a case of a Freeman-Sheldon syndrome in 12-year-old girl correction of undergoing kyphoscoliosis under somatosensory evoked potential monitoring. She had a characteristic appearance of Freeman-Sheldon syndrome such as hypoplastic alae nasi, high narrow palate, marked microstomia with pursed lips and clenched fingers. On arriving at the operating room, she was intubated by awake nasotracheal intubation with fiberoptic bronchoscopy and anesthetized with propofol and fentanyl.