Williams syndrome, initially described by Williams et al. in 1961 is associated with characteristic dysmorphic features, congenital heart disease, distinctive behavior, and emotional traits. Sudden death has been reported perioperatively due to congenital heart disease in patients suffering from Williams syndrome. We present a case of a patient with Williams syndrome underwent preauricular fistulectomy and medial rectus recession. Airway management and tracheal intubation were successfully performed on the patient. For anesthetic management, sevoflurane and vecuronium, as well as sevoflurane and rocuronium were employed during the first and second operation, respectively. Anesthetic managements were performed uneventfully in this patient.