Background: Kabuki syndrome (KS) is a rare congenital disorder associated with craniofacial and airway abnormalities that may complicate anaesthetic management. Case Report: A 9-year-old girl with KS underwent planned adenoidectomy with bilateral grommet insertion. Despite a reassuring preoperative airway assessment, intraoperative examination revealed a bifid uvula, submucous cleft palate, and posterior septal defect. Due to the risk of postoperative velopharyngeal insufficiency, adenoidectomy was abandoned, and bilateral grommet insertion was completed. Intubation was successfully performed using video laryngoscopy. Conclusion: Careful preoperative airway and palatal assessment is essential in KS. Recognition of occult abnormalities and close anaesthetic-surgical collaboration can significantly influence surgical planning and improve perioperative safety.