Autoimmune liver diseases are chronic inflammatory conditions leading to an etiologically undefined immunemediated attack aimed at hepatocytes and the biliary epithelium. Drugs used in autoimmune liver disease such as ursodeoxycholic acid, prednisolone and azathioprine are not effective in all patients, therefore, new therapeutic approaches are needed for autoimmune liver diseases that are refractory to standard therapy. Biotherapy is a thriving area of research and development, and is used in the treatment of chronic autoimmune liver diseases. However, to date, there is no clinically validated standard biotherapy for autoimmune liver diseases. Thus, future clinical trials are required to evaluate the effectiveness and safety of biotherapy before this approach can be used in routine clinical practice for the therapy of autoimmune liver diseases. This article provides an overview of emerging biotherapy for autoimmune liver diseases.