PURPOSE OF REVIEW: This review highlights recent advances in understanding the regulation of bile acid transport in cholestasis and the pathogenesis and treatment of a variety of cholestatic conditions. RECENT FINDINGS: Highlights include new understanding of the role of Mrp4 in bile acid homeostasis in cholestasis, new insights into the pathogenesis of specific cholestatic syndromes including primary biliary cirrhosis, primary sclerosing cholangitis, biliary atresia, and progressive familial intrahepatic cholestasis, and clinical trials of therapies for primary biliary cirrhosis, primary sclerosing cholangitis and intrahepatic cholestasis. SUMMARY: Our understanding of the molecular mechanisms of cholestasis is advancing. These advances will hopefully lead to more effective therapies for specific cholestatic conditions.