Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver caused by an abnormal autoimmune response of hepatocytes. The incidence of autoimmune hepatitis is 0.1-1.9/100,000 per year, and is present in 11-23% of all patients with chronic liver disease. The etiology of AIH is currently unknown, but many environmental, genetic, and pathophysiological factors are thought to be involved in its development. the pathogenesis of AIH implicates a lack of tolerance to liver autoantigens in susceptible individuals. It is well established that there is an autoimmune response targeting hepatocytes in AIH, which is highly correlated with other autoimmune diseases such as type I diabetes, Graves’ disease and proliferative glomerulonephritis. The diagnosis of AIH is dependent on histological abnormalities, clinical features and biochemical findings, including abnormal plasma globulin levels and the presence of one or more characteristic autoantibodies such as ANA, SMA and anti-SLA. The presence of a large number of plasma cells in the infiltrates of interfacial hepatitis is a characteristic histological feature of AIH. The simplified diagnostic criteria have high sensitivity and specificity for the diagnosis of AIH in Asian patients. The original revised diagnostic criteria have a complementary role in avoiding negative misdiagnosis in patients with atypical AIH. Despite the lack of epidemiological data, AIH is heavily diagnosed in China and is considered a major cause of non-viral chronic hepatitis. Increasingly, liver biopsy and plasma autoantibody test results are routinely reported in liver disease centers. Because hepatologists and pathologists are alert and aware of the histologic features of AIH, an increasing number of patients in our medical centers who were previously thought to be cases of chronic hepatitis of unknown cause are being diagnosed with AIH. Fortunately, AIH can be effectively controlled with current therapeutic approaches. Immunosuppressive therapy can reduce liver inflammation and reverse the liver fibrosis process, ultimately improving the patient’s prognosis and quality of life. AIH responds well to immunosuppressive therapy, although patients with advanced or combined severe disease are unlikely to achieve remission. Liver transplantation is the last option for patients with decompensated end-stage AIH. Transplantation of immunomodulatory cells, such as regulatory T cells, may become a promising treatment in the near future. Paying attention to this disease, its clinical presentation, treatment and progression is important for hepatologists, as many of these cases have been overlooked in the past.