Abstract
HUWE1-related neurodevelopmental disorder is an extremely rare X-linked condition with fewer than 100 cases reported worldwide and no established perianesthesia guidelines. We report the successful anesthetic management of an 8-year-old girl with this disorder undergoing dental surgery. Her clinical picture was complicated by profound intellectual and physical impairment, blindness, and pharmacoresistant Lennox-Gastaut syndrome managed with a 3.5:1 ketogenic diet. A multidisciplinary approach focused on mitigating risks of hypotonia, epilepsy, and metabolic disruption led to an uneventful outcome. Key interventions included quantitative neuromuscular monitoring with sugammadex reversal, glucose-free fluid management, and parental presence as a primary non-pharmacological anxiolytic strategy. This report provides a practical, collaborative framework for managing the complex perioperative challenges in this unique patient population, highlighting the essential role of peri-anesthesia nursing in ensuring safety and patient comfort.