Autoimmune hepatitis (AIH) is an inflammatory process, where autoantibodies attack and damage liver cells. Like many autoimmune diseases, aetiology is unknown and it affects females more than males. It is characterised by both the presence of autoantibodies in serum and by high activity hepatic infiltrate pathohistology. There are two types of pediatric AIH identified; type I and type II. The types are differentiated on serological basis mainly: type I is positive for smooth muscle antibody (SMA) and/or antinuclear antibody (ANA); whereas, type II is positive for antibody to liver kidney microsome type 1 (ALKM1). Moreover, type II clinical presentation is more acute, usually at younger age as well as lower levels of IgA. In comparison to type I pediatric AIH, often present at older age/puberty. Nevertheless, the response to treatment and prognosis are comparable in both types. Genetic predisposition based on HLA typing has been found among different ethnicities. The treatment for both types is long- term immunosuppression classically with steroids and azathioprine; the treatment aims to normalise serology and liver histology – remission. Non-classic treatment is reserved for patients that are resistant to classic treatment; i.e. immunosuppression with budesonide, mycophenolate mofetil and cyclosporine. Relapses can occur; complete remission without the need of long-term immunosuppression is only achieved in 1 in 5 patients. Pediatric AIH has shown a significant association with other autoimmune diseases like autoimmune sclerosing cholangitis, inflammatory bowel disease, autoimmune thyroiditis, psoriasis and rheumatoid arthritis; some share clinical features and are collectively called overlap syndrome.