We aimed to study the clinical profile and etiology of Acute Encephalitis Syndrome (AES) in a tertiary care centre of western India.All patients evaluated by the neurology department, who fulfilled the standardised case definition for encephalitis (given by International Encephalitis Consortium) over 2 year period were screened. Routine laboratory investigations, CSF, Neuroimaging and EEG were done in all patients. Short term follow-up of one month was done to see the outcome.: Out of total 85 patients of AES seen over two years; viral etiology was identified in 26 (30.5%) patients; 8 (9.4%) patients had autoimmune cause and in 53 (62.4%) patients, no specific ethology could be found. Long duration of symptom onset to hospitalisation, seizures, abnormal behaviour, involuntary movements (automatism, dyskinesia, or dystonia), and autonomic dysfunction favours a diagnosis of autoimmune encephalitis. At one month, good outcome (mRS <2) was seen in 51(60%) patients and 34 (40%) patients had a poor outcome (mRS > 2), out of which 29(34.1%) patients expired. Viral encephalitis was the commonest cause of AES; followed by autoimmune encephalitis. Specific cause could not be ascertained in large number of patients, even after extensive evaluation. AES patients had prolonged hospital stay and significant morbidity and mortality.