Propofol is used for the treatment of refractory status epilepticus. When given as a long-term infusion, in combination with catecholamines and/or steroids, propofol rarely causes the fatal complication, known a propofol infusion syndrome. Herein, the case of a previously healthy 8-years-old boy with status epilepticus is reported. He was treated with propofol, in doses ranging from 8.6 to 10.9 mg/kg/h, for 43 hours. The propofol resulted in multiple organ failure, with rhabdomyolysis, which caused the death of the patient. This case fulfills all the criteria of propofol infusion syndrome.