There have been no case reports to date describing the technical aspects of tracheal intubation in a patient with a goitre associated with McCune-Albright syndrome (MAS), even though goitre is frequently observed in this condition. I describe a case of resection of a giant goitre in a patient with MAS, with difficult airway management. Preoperative investigation showed that the trachea was shifted to the right by the goitre, with the narrowest part of the tracheal lumen 4 mm in diameter. There was dome-shaped protuberance of the posterior pharyngeal wall into the airway. The patient had an S-shaped total spine, a short neck, and a relatively large jaw, which interfered with airway visualisation during intubation. Anaesthesia was induced with light sedation and supplemental oxygen. Endotracheal intubation was successfully performed using a fiberoptic laryngoscope and a flexible, spiral-wound, obtuse-tipped tracheal tube.