Endoscopic therapy for biliary tract disease in patients with end-stage liver disease (ESLD) before liver transplantation is safe and effective. Reported results in patients with choledocholithiasis, primary sclerosing cholangitis (PSC), and symptomatic gallbladder diseases are encouraging. Prompt recognition and appropriate treatment of symptomatic gallbladder and bile duct disease are important in reducing morbidity and mortality in these high-risk patients while they await liver transplantation. Confirmation of tissue diagnosis of cholangiocarcinoma in patients with sclerosing cholangitis is still difficult. Better screening tools and diagnostic methods are necessary for early detection. Because liver transplantation is the only definitive therapy for patients with advanced cirrhosis, maintenance of their candidacy with either endoscopic or radiologic therapeutic interventions is warranted until transplantation. Endoscopic therapy is the preferred method when feasible. If necessary, percutaneous transhepatic biliary drainage (PTBD) is a viable alternative because both avoid the attendant risks of surgery in a high-risk population with advanced liver disease.