The Farnesoid X nuclear receptor (FXR) is a nuclear receptor of bile acids whose activation suppresses the synthesis of bile acids stimulates their excretion in the bile and inhibits its uptake in hepatocytes. FXR is also involved in the regulation of over 250 genes including those responsible for the control of lipid and carbohydrate metabolism. The activation of FXR also induces anti-inflammatory effects and antifibrotics. Over the past 10 years they have been synthesized and studied various FXR agonists which have demonstrated beneficial effects in the treatment of the main pathologies cholestatic diseases including primary biliary cholangitis, cholangitis primary sclerosing and cholangiocarcinoma.