Background: Status epilepticus is defined as a neurologic emergency in which there is at least 5 minutes of continuous seizure activity or recurrent seizures with a return to baseline between seizures. The aim of this research was evaluation of clinical and etiological profile of refractory status epilepticus (RSE) among children. Material and methods: The study was carried out between 2017 – 2021. All children have presented convulsive status epilepticus (SE), subsequently with development of RSE (refractory status epileptic). We try to identify the main characteristics of children with RSE and those without an evolution of RSE. Results: Fifty-five children, out of whom 32 boys with SE were enrolled in the study, of which 20 children (36%) developed RSE. Central nervous system (CNS) infections were the most common causes of SE and development of RSE (51% of SE and 53% of RSE, p > 0.05). Noncompliance of antiepileptic medication served as the second cause for evolution of RSE. The overall mortality rate was 10.9%, the chances of death in case of RSE (20%) being higher than in case of SE (5.7%). The unfavorable prognosis was seven times higher in children with RSE, compared to children who developed SE (PR = 7.0; 95% CI:1.6 – 22.3). Conclusions: In the management of CNS infections the possibility of developing RSE should be considered and promptly managed in an intensive care unit in order to reduce the risk of mortality and morbidity of this severe neurological condition.