Propofol infusion syndrome (PRIS) is a fatal syndrome associated with continuous propofol infusion, which causes several serious clinical conditions such as rhabdomyolysis, acute kidney injury (AKI), lactic acidosis, and hyperlipidemia. We report the case of a 44-year-old adult male patient suspected of having PRIS who was successfully treated by promptly discontinuing propofol infusion. The patient underwent total arch replacement for Stanford type A aortic dissection. Propofol was used for postoperative sedation, but his serum CK level was elevated up to 15,247 IU/l. He also developed AKI and lactic acidosis. Therefore, we strongly suspected PRIS, so propofol was discontinued. His serum CK level subsequently decreased, and AKI and lactic acidosis improved rapidly. CT scan showed some high-density areas in the hip and femoral muscles, which can be considered post-rhabdomyolysis changes. Serum CK, pH, and lactate levels should be measured routinely during prolonged propofol infusion, and alternative sedatives should be administered promptly if PRIS is suspected.