Propofol infusion syndrome (PRIS) is a serious complication that occurs during propofol infusion. PRIS is a fatal clinical condition that includes hepatomegaly, fatty liver, hyperlipemia, metabolic acidosis, rhabdomyolysis and myoglobinemia. PRIS is possibly developed by the lowered ATP production due to the inhibition of mitochondrial function and fatty acid metabolism. PRIS appears more commonly in children because of their property that metabolism and elimination of propofol are prolonged, according with high dependency on fat metabolism. Safe application of propofol has been indicated as the precise mechanism of PRIS has been clarified.