Autoantibodies against nuclear or cytoplasmic components are serological hallmarks that reflect an autoimmune response. These autoantibodies are produced by the antigen-driven immune system. The detection of autoantibodies may be helpful in the diagnosis of autoimmune liver diseases including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Current studies are changing our understanding of autoantibodies in liver diseases. As a result, AIH has been rearranged into two major subtypes. Novel serological tests are applied into patients with AIH, and the clinical validation of these tests has been described. Several recent studies that antinuclear antibodies including antibody to gp210 and anticentromere antibody seemed to have prognostic values in patients with PBC were reported. Current articles provide the evidence that autoantibodies are present even in patients with non-alcoholic steatohepatitis (NASH) as well as in patients with autoimmune liver diseases. Experimental animal models for AIH have been established. These murine models are useful for analyses of genetic determinants.