Objective To investigate the clinical characteristics and prognosis of refractory status epilepticus (RSE) patients admitted to the pediatric intensive care unit (PICU). Methods Clinical data of children with RSE admitted to the PICU from January 2017 to December 2021 were retrospectively collected, and they were divided into survival and death groups based on survival status. Gender, age, body mass index, etiology, Glasgow Coma Score (GCS) at admission, head imaging, electroencephalogram, antiepileptic drugs, and modified Rankin Scale (mRS) at discharge were summarized. The clinical characteristics, treatment and prognosis of children with RSE were analyzed. Results A total of 80 children with RSE were admitted, 39 (48.8%) were male, median age at onset was 38 (12-80.8) months, including 12 (15%) cases of super-refractory status epilepticus sustained (SRSE).The causes of RSE were severe encephalitis in 50 cases, epileptic syndromes in 9 cases, intracranial tumors/haemorrhages in 7 cases, immune encephalitis in 4 cases, hereditary-metabolic encephalopathy in 4 cases, septic encephalopathy in 3 cases, and septic meningitis in 3 cases. Mechanical ventilation was required in 53 cases (66.3%), and the median duration of mechanical ventilation was 96 (54-239) h. Seventy-seven cases were examined by cranial magnetic resonance imaging, of which 61 cases (79.2%) were abnormal. The median duration of convulsive seizures was 19 (2~46) h, the median length of stay in the PICU was 8.5 (5-17.8) d, and there were 19 deaths (hospitalization morbidity and mortality rate of 23.8%). 61 patients survived to be discharged with a good recovery of neurological function (mRS < 3) in 23 cases (37.7%). Comparing the survival and death groups, there was a significant difference in the proportion of admission GCS scores and imaging abnormalities (both P<0.05). Conclusion The etiology of new-onset RSE in this group of children was mainly severe encephalitis, and the GCS scores and cranial imaging results on admission were of reference value for prognostic judgment.