Dravet syndrome (DS) is a rare and severe form of epilepsy that begins in infancy. This is particularly burdensome because repeated epileptic seizures lead to cognitive decline. We describe the case of a 12-year-old girl who was diagnosed with DS and was scheduled to have gingival reduction around her mandibular molars. Despite the patient being intellectually disabled, she was able to cooperate somewhat during medical procedures, including intravenous cannulation. Under the assumption that the major problem with anesthesia for DS would be the regulation of body temperature–induced seizures, we used body temperature management equipment to maintain the patient's body temperature during the procedure. We opted for intravenous sedation and administered a total dose of 4.5 mg midazolam throughout the procedure. Anesthesia was completed within 1 hour and 20 minutes without any adverse events. To the best of our knowledge, no previous studies have documented the anesthetic management of DS. In this case, no adverse events occurred perioperatively. However, the patient's temperature rose to that which indicated a slight fever despite the use of a standard cooling technique.