Propofol infusion syndrome has been reported as a fatal syndrome described in critically ill patients undergoing longterm propofol infusion at high dose. To date 21 pediatric cases and 15 adult cases have been described, but there has been no report of this syndrome in Japan. The main clinical symptoms of this syndrome are cardiac failure, fatal arrhythmia, rhabdomyolysis, severe metabolic acidosis, hyperkalemia et cetera and they occur suddenly. The pathogenesis of this syndrome is considered mutual action between long-term propofol infusion at high dose, critical illness and the use of catecholamines or steroids. We present a case of an adult patient who was suspected propofol infusion syndrome. A 28-years old man was admitted to the ICU because of refractory status epilepticus. While he received propofol infusion of more than 5.75mg·kg^-1·hr^-1 for 56.5 hours dose for the treatment of status epilepticus, suddenly he developed circulatory failure (cardiac failure), rhabdomyolysis, severe metabolic acidosis, hyperkalemia, fatal arrhythmia (ventricular tachycardia and ventricular fibrillation), which resulted in death.