BACKGROUND: This case report described the anesthetic management and postoperative challenges of a patient diagnosed with Congenital Contractures of the Limbs and Face, Hypotonia, and Developmental Delay (CLIFAHDD) syndrome who underwent tenomyotomy of the right hand tendons. This syndrome, characterized by facial dysmorphisms, musculoskeletal abnormalities, and respiratory disturbances, including central apnea, is caused by a mutation in the NALCN gene. PURPOSE: A 9-year-old girl diagnosed with CLIFAHDD syndrome underwent tenomyotomy of the right hand tendon. General anesthesia was administered. Despite stable intraoperative conditions, the patient experienced inadequate ventilation with multiple episodes of apnea after extubation, necessitating assisted ventilation using a face mask. CONCLUSIONS: This case highlights the challenges and considerations in the anesthetic management of patients with CLIFAHDD syndrome, emphasizing the need for tailored approaches and vigilant postoperative monitoring to mitigate the potential respiratory complications associated with this rare genetic disorder.