Introduction: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a immune mediated encephalitis characterised by a combination of psychiatric illness followed by lowered levels of consciousness, hyperkinetic movements disorders, seizures, speech abnormalities. In this study, we have evaluated the clinical profile, treatment and outcome of 23 CSF anti-NMDAR antibody and 1 serum anti-NMDAR antibody confirmed cases. Materials and Methods: All the consecutive patients presented with neurological manifestations who were positive for  either CSF or serum anti-NMDAR antibody in Department of Pediatrics Government Medical College, Trivandrum, a tertiary care hospital from January 2014 to July 2018 were included in the study. We excluded patients whose serum and CSF was negative for anti-NMDAR antibody. Results:Twenty four children (males-2,females-22) were included in the study. Common prodromal symptoms observed was fever, vomiting. Majority of patients presented with behavioural problems (n=13) in the form of insomnia, fear, hallucinations, irritability. Seizures (n=11) were the second commonest presentation with 2 patients presenting with status epilepticus. Movement abnormalities (n=5) observed were oro-facial dyskinesia, choreoathetoid movements, dystonia. Complete mutism was seen in 17 patients. One patient presented with elevated CPK levels and urine myoglobinuria currently receiving treatment. All patients received first line therapy (both methyprednisone and IV immunoglobulin) and majority(n=20) received second line therapy (Rituximab, Cyclophosphamide, plasma exchange, Azathioprine, Mycophenolate Mofetil. Best response was seen with Rituximab in terms of cognitive and motor outcome. Conclusion: Most of the patients in our study improved after Rituximab therapy. Rituximab can be considered as first line therapy in severe anti-NMDAR encephalitis cases