Background: Kabuki syndrome is a rare developmental disorder characterized by distinctive craniofacial features and variable multisystem involvement. Its anesthetic implications extend beyond potential difficult intubation to respiratory vulnerability, swallowing dysfunction, cardiovascular abnormalities, and neurological comorbidities. These concerns are particularly relevant during upper gastrointestinal endoscopy, when the endoscopist and anesthesiologist share access to the upper airway. Clinical illustration: A boy with a reported pathogenic KDM6A variant underwent esophagogastroduodenoscopy for investigation of dysphagia. His phenotype included a high arched palate, axial hypotonia, developmental delay, and a history of seizures treated with valproate. Previous echocardiography showed no structural cardiac abnormality. The clinical presentation and procedural photographs provide an opportunity to revisit the anesthetic implications of this uncommon syndrome. Discussion: This narrative review addresses airway assessment, aspiration risk, respiratory depression, neuromuscular recovery, associated organ disease, and preparation for rescue interventions. Published anesthetic experiences demonstrate heterogeneous clinical presentations and support individualized planning. Concerns about malignant hyperthermia and latex allergy should be interpreted according to the strength of the available evidence and the individual history. Conclusion: Recognition of Kabuki syndrome should prompt a phenotype based anesthetic assessment even for brief diagnostic procedures. During upper gastrointestinal endoscopy, coordinated airway management and attention to dysphagia, hypotonia, and recovery are central to perioperative planning.