Anesthetic management for a patient with mitochondrial myopathy is described. A 57 year-old-female underwent laparoscopic cholecystectomy for cholelithiasis. The patient had been diagnosed as having mitochondrial myopathy from muscle biopsy. Anesthesia was managed with total intravenous anesthesia with propofol, fentanyl, and ketamine. Her reaction to vecuronium bromide was within normal limits evaluated with a neuromuscular activity parameter, train-of-four ratio. No serious acidosis, hyperlactemia, hypothermia, nor prolonged recovery from the anesthesia was observed. As inhaled anesthetics may be contraindicated for mitochondrial myopathy, and nitrous oxide for laparoscopic surgery is relative contraindication, total intravenous anesthesia with muscle relaxant titration is appropriate for laparoscopic surgery for patients with mitochondrial myopathy.