Propofol-related infusion syndrome (PRIS) is a rare but fatal complication. Unexplained metabolic acidosis, rhabdomyolysis, hyperkalemia, myocardial dysfunction, cardiovascular collapse and acute kidney injury are the main characteristics of PRIS. Herein, we report a case of PRIS in a neurosurgical adult patient, who had received high-dose propofol continuous infusion in order to control intracranial pressure in an intensive care unit. She manifested severe metabolic acidosis, rhabdomyolysis, acute kidney injury and myocardial dysfunction. As soon as PRIS was diagnosed, propofol infusion was stopped. Conservative treatments, such as vasopressors and inotropics, continuous renal replacement therapy and extracorporeal membrane oxygenation were used to treat PRIS. However, she finally expired. This case report suggests that a great caution to PRIS is needed in a situation with high-dose propofol continuous infusion.