Background: This study was aimed to evaluate the effectiveness of midazolam (MDZ) infusion and topiramate (TPM) add-on treatment for refractory status epilepticus (RSE) and their treatment outcome in children. Methods: Nineteen children were involved and their clinical data were retrospectively analyzed. After failure of initial conventional treatment, all were given midazolam infusion, titrated every 15 minutes to the clinical response or maximum dose of 20 mcg/kg/minutes. Eleven out of them were given additional TPM started at 1-2 mg/kg/day by nasogastric tube and titrated to the favorable response every 1-3 days. We subdivided them into 2 groups by whether TPM added (TPM group) or not (MDZ group) and compared them each other. Results: The age of subjects ranged from 2 months to 14.6 years (54.9 ± 53.3 months; male 10, female 9). The duration of initial seizures ranged from 30 minutes to 24 hours (218.4 ± 432.9 minutes). With respect to the type of seizures, 84 % were convulsive and 16 % were non-convulsive. In the MDZ group (4.0 ± 2.9 mcg/kg/minutes of MDZ), time to seizure control ranged from 1 day to 2 days (1.1 ± 0.4 days), but seizures recurred later on in 50 % of them. In the TPM group (6.3 ± 1.5 mg/kg/day of TPM), time to seizure control ranged from 1 day to 5 days (1.9 ± 1.5 days), but seizures recurred in 36 % of them. There was no statistical difference between them. One (5%) died of sepsis and seven children (37%) experienced cardio-respiratoty compromise. Conclusion: The results indicate that midazolm infusion is still the mainstay of treatment, but TPM add-on treatment may be another option for RSE. However, further studies are needed to establish its precise role and safety.