Background: Autoimmune hepatitis is a chronic liver disease that could lead to cirrhosis, hepatic insufficiency, and even death in case of delayed diagnosis or improper treatment. It has a wide range of clinical, paraclinical and histological manifestations. Our aim was to determine the clinical and paraclinical manifestations and response to treatment among Iranian patients with autoimmune hepatitis.Materials and methods: Thirty-one patients with autoimmune hepatitis, referred to hepatology clinic of Taleghani & Day hospitals were included according to the criteria of International Autoimmune Hepatitis Group. Score of 2:15 was considered as definite diagnosis. Initial data including history, physical examination, age, sex, liver function tests, electrophoresis, serologic tests, pathologic reports, treatment and response to treatment were all determined.Results: The study population included 22 females and 9 males with the mean age (SD) of 34::1:y:2§ars. The most common clinical manifestations were fatigue, jaundice and menstrual disturbance respectively. 38% of patients were ANA positive and 54.8% ASMA positive. Of22 patients undergone liver biopsy, 6 showed cirrhosis. Among non-cirrhotic patients the mean histologic grade was 6.5 and stage was 3. Mean duration of complete response was one year. Response to treatment and remission phase was longer in cirrhotic patients Conclusion: Autoimmune hepatitis in Iranian patients and response to treatment resemble that in western countries, however, serologic criteria differed. It seems as if impaired laboratory techniques and lack of sufficient kits are the reason for such difference.