We describe the anesthetic management of a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, stroke-like episodes (MELAS) syndrome, and a form of mitochondrial myopathy. A 14 year-old-male with MELAS syndrome underwent tracheostomy under general anesthesia. Anesthesia was managed with thiopental sodium, nitrous oxide, sevoflurane, and rocuronium. His reactions to anesthetics and muscle relaxant were within normal limits. No serious complication was observed. The implication of MELAS syndrome for anesthesia are discussed.