Autoimmune hepatitis (AIH) is a chronic and progressive disease. The etiology and epidemiologic characterizations of AIH are not fully understood. It has a prolonged course and can affect all age groups. Symptoms and signs may appear years before diagnosis, and may be manifested for extended periods only as minor liver enzymes abnormalities. Clinical manifestations vary from lethargy and jaundice, to extremely severe deterioration in quality of life, hepatic failure and liver transplantation or death. In most cases there is a good response to therapy, making it important to diagnose the disease early in its course. The objectives of this review are to increase the awareness to AIH, to the diagnostic methods and to the treatment modalities and to discuss updates regarding AIH.