Propofol is increasingly used for the treatment of status epilepticus due to the ease of use and tolerability, even if safety data from randomized clinical trials are lacking. An association of high infusion rates of propofol (>5 mg/kg/h) for more than 48 h and constellation of acidosis, rhabdomyolysis, and cardiovascular collapse has been reported in children, but has only been described in a few adult cases. We report a case and autopsy findings of an adult who developed rhabdomyolysis and cardiac failure after receiving propofol for status epilepticus. The patient became symptomatic within 55 h after initiation of propofol infusion. The maximal infusion rate did not exceed 7.2 mg/kg/h, and propofol in excess of 5mg/kg/h was infused for less than 20 h. Preexisting antiepileptic medication may have exacerbated acidosis. Propofol infusion for the treatment of status epilepticus should be carefully weighted against its real risk to develop propofol infusion syndrome, and alternative agents such as benzodiazepines or barbiturates should be considered for first line therapy. If necessary, prolonged propofol infusion at high doses for the treatment of status epilepticus should be used with caution, and in all cases careful monitoring for rhabdomyolysis and acidosis must be performed.