The McCune-Albright syndrome is rare disease diagnosed by the clinical triad, fibrous dysplasia, cafe-au lait skin pigmentations and endocrine hyperfunction. Those patients with bone issues could have various surgeries under general anesthesia. Airway abnormality and various endocrine abnormalities should be considered during general anesthesia for McCune-Albright syndrome patients. A 15-year-old male with McCune-Albright syndrome was admitted with complaint of right nasal obstruction originated from fibrous dysplasia. Endoscopic resection of nasal cavity lesion was scheduled under navigation system guidance. Difficult airway could be anticipated due to protrusion of maxilla and right nostril. Awake fiberoptic intubation was performed by spray-as-you-go technique. When an anesthesiologist expects to take care of the patient with the McCune-Albright syndrome, the most appropriate anesthetic induction and tracheal intubation technique should be selected, and multiple backup instruments such as supraglottic device, video laryngoscope and fiberoptic bronchoscopy should be prepared.
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