Autoimmune hepatitis (AIH) is a rare chronic disease of the liver with an excellent prognosis under medical therapy capable of reaching complete remission. The diagnosis of AIH relies on the exclusion of viral, metabolic, genetic and toxic aetiologies of chronic hepatitis, or hepatic injury. Autoantibodies contribute to the diagnosis of AIH and have led to the serological subclassification into three distinct types. Also, immunogenetic associations suggest heterogeneity of the syndrome of AIH. Treatment is not based on serological types but is uniformly employed for all subtypes of AIH. Although 90% of patients respond to treatment, immunosuppressive drugs used in transplant medicine have been employed for patients with treatment failure. New drugs, such as budenoside, are being evaluated for the long-term treatment of AIH with a reduction in steroid side-effects. Liver transplantation is an established treatment option for patients who fail to reach remission and progress to cirrhosis and liver failure. In Europe, about 4% of cirrhotic patients with the diagnosis of AIH undergo transplantation. The diagnosis and awareness of the disease is designed to reduce mortality and morbidity.