Carnitine palmitoyltransferase（ CPT） II deficiency, a very rare inherited disorder of mitochondrial fatty acid oxidation, is characterized by myalgia attacks leading to rhabdomyolysis, hyperkalemia, and acute renal failure. Here, we report an anesthetic management in an infant patient with CPT II deficiency. A 16-month-old child underwent implantation of a totally implantable central venous access device. General anesthesia was performed with sevoflurane, fentanyl, and rocuronium. No complications occurred during the perioperative period. In patients with mitochondrial fatty acid oxidation disorders such as CPT II deficiency, it is important to ensure continuous glucose infusion with blood sugar monitoring. Avoiding drugs associated with rhabdomyolysis and maintaining normothermia are keys for safe anesthetic management.