Background: Rasmussen encephalitis (RE) is a chronic, progressive encephalitis affecting one hemisphere of the brain. Intractable focal seizures, progressive neurological & cognitive decline and hemispheric atrophy are common clinical and radiological presentations of the disease. Objective: To see the clinical spectrum & short-term treatment outcome of Rasmussen Encephalitis. Method: It was a prospective interventional study, conducted at Department of Pediatric Neurology, Bangabandhu Sheikh Mujib Medical University, Dhaka from July 2022 to July 2023. Total 15 patients with Rasmussen encephalitis were evaluated after IV Methylprednisolone therapy at the doses of 20-30 mg/kg/day. Results: Among 15 patients, 8 (53.3%) were aged 5–10 years and 7 (46.7%) were <5 years; males predominated (11, 73.3%). All presented with seizures, hemiparesis, neuroregression, and cognitive impairment. Dysarthria was observed in 10 (66.7%) and facial nerve palsy in 4 (26.7%). Focal seizures were most common (7, 46.7%), followed by generalized tonic–clonic seizures (3, 20%). EEG showed unihemispheric slowing in 12 (80%) and generalized slowing in 3 (20%). Neuroimaging revealed unihemispheric insular–perisylvian atrophy with basal ganglia involvement in all cases. Following IV methylprednisolone, seizure frequency improved in 13 (86.7%) and EEG improved in 8 (53.3%). Conclusion: All patients with Rasmussen encephalitis presented with seizure, hemiparesis, neuroregression & cognitive impairment. IV Pulse methylprednisolone therapy were effective where seizure frequency reduced more than three-fourth cases & electroencephalographical improvement occured more than half of the cases of all Rasmussen encephalitis.