The definition of refractory status epilepticus (RSE) is the state of epilepticus that persists for longer than 60 minutes despite an adequate dose of benzodiazepine and an antiepileptic drug. Topiramate is a broad-spectrum antiepileptic drug used as a monotherapy as well as an adjunctive therapy in the treatment of epilepsy. We encountered a 32-month-old girl with RSE, who showed spastic quadriplegia, hydrocephalus and a previous history of 3 times recurrence of status epilepticus in the presence of global development. Although the girl was treated with lorazepam, phenytoin, phenobarbital and even with midazolam, seizure lasted for more than 60 minutes. Through pentobarbital coma, seizure was controlled clinically on the state of EEG. However, it recurred. Therefore, the patient was treated with an overdose amount of topiramate (10 mg/kg/day) by nasogastric tube without titration as an add-on therapy and then treated with a reduced amount of topiramate (4 mg/kg/day). Finally, the seizure became controlled.