Biliary cystic disease, though uncommon, can present at a wide range of ages with a wide range of symptoms. Choledochal cysts are associated with the development of both cholangiocarcinoma and gallbladder cancer. Thus, most biliary cystic disease is best managed operatively. Many factors should be considered when performing surgery on patients with choledochal cysts, including age, presenting symptoms, cyst type, associated biliary stones, prior biliary surgery, intrahepatic strictures, hepatic atrophy/hypertrophy, biliary cirrhosis, portal hypertension, and associated biliary malignancy. When feasible, surgical treatment should consist of cholecystectomy and complete surgical excision of extrahepatic cysts with Roux-en-Y reconstruction. Because the risk of recurrent cholangitis is significant and additional symptoms and problems are common, the use of long-term soft Silastic® biliary stents (Dow Corning Corp., Midland, MI) should be considered when complex intrahepatic and extrahepatic cystic disease is present. Alternatively, the Roux-en-Y jejunal limb can be marked at the fascia for future percutaneous access. Reconstruction via hepaticoduodenostomy and jejunal interposition has been associated with increased postoperative pain due to bile reflux gastritis. Thus, hepaticojejunostomy reconstruction is recommended. For choledochal cysts involving the distal bile duct, the bile duct should be excised at the intrapancreatic portion. Resection of the pancreatic head should be reserved for patients with established malignancies. Surgical excision of the intrahepatic portion of the bile duct should be individualized to include preservation of hepatic parenchyma when the liver is not cirrhotic. If cirrhosis is advanced, hepatic transplantation may be indicated, but this is rare. Oncologic principles should be followed in the presence of a malignancy. Lifelong follow-up is required because of the possibility of a "field" defect increasing susceptibility to cancer throughout the biliary tract epithelium. Copyright © 2006 by Current Science Inc.