We are reporting two cases of SE with lethal rhabdomyolysis. Both were treated according to the guidelines on antiepileptic drug management by the Danish Paediatric Society, with one and two hours' delay, respectively. Intubation and midazolam infusion were needed to control seizures. After approximately one day both developed severe rhabdomyolysis with progressive DIC and acute renal failure, causing death despite intensive care, including acute hemodialysis. The aim of this case report is to emphasise one of the rare but severe consequences of SE and also to suggest that patients with known repetitive SE may benefit from written individual treatment schedules to avoid wasting time.