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A 12-year-old boy with mitochondrial encephalomy- opathy underwent pacemaker implantation for com- plete atrioventricular block. He was hospitalized as his general condition deteriorated. Furthermore, Holter electrocardiogram revealed rapid atrioventricular con- duction defect Anesthesia was induced with propofol, fentanyl, and rocuronium and maintained with continuous infusion of propofol and remifentanil with administration of fen- tanyl and rocuronium under neuromuscular monitoring during surgery. Bispectral index was monitored and maintained at approximately 40. He could not commu- nicate and had unstable circulation. Therefore, we pro- longed the anesthesia induction time. In addition, for the purpose of decreasing the amount of anesthetic required, an ultrasound-guided transversus abdominis plane block was performed. Throughout the periopera- tive period, neither cardiovascular instabilities nor pro- gression of metabolic acidosis and sudden body tem- perature increases were observed. Many important points must be considered when administering anesthesia to a child with mitochondrial disease. When we plan the anesthetic strategy, moni- toring, and so on properly, the appropriate anesthesia management can be performed.