Bile duct and gallbladder cancer are relatively uncommon. Predisposing factors include primary sclerosing cholangitis and gallstones larger than 3 centimeters. Patients present with signs of biliary obstruction and cholestasis. A serum CA 19-9 elevated above 100 U/mL is a useful marker. The diagnosis is implied on imaging studies and confirmed by tissue obtained at endoscopic retrograde cholangiopancreatography or surgery, or by the clinical course. Surgery is the only curative therapy and survival is improved with resection of early stage disease. Endoscopic or percutaneous transhepatic stenting provides effective palliation. Generally, survival is less than 1 year.