Primary sclerosing cholangitis is a cholestatic liver disease strongly associated with IBD. Considerable advances in the understanding of its pathogenesis have been made. The idea of autoimmunity affecting genetically susceptible individuals is largely accepted; however, much remains to be explained about the origin of this disease. Despite active investigation of different therapeutic modalities with the goal of modifying disease progression, liver transplantation continues to be the only option to provide survival benefit in these patients.