We present a 12-year-old female Klippel-Feil syndrome patient who underwent a surgical correction of thoracolumbar scoliosis. The patient had congenital fusion of the cervical vertebrae (C2,3,4,5,6) and showed the classic triad consisting of a low posterior hairline, short neck, and neck motion limitation. Because these patients may have a risk of neurological damage during airway management and subsequent positioning, anesthesiologists need to take care regarding the cervical spine pathology and degree of instability. In this case, fiberoptic bronchoscope guided intubation was performed. We discuss the anesthetic management of Klippel-Feil syndrome patients with a review of the relevant literature.