Choledochal cysts are congenital cystic dilations of the extrahepatic biliary ducts. Intrahepatic cystic dilation can coexist (Caroli's disease), but it represents a distinct problem and is managed differently. Patients may present with symptoms at any age, but the classic triad of epigastric pain, abdominal mass, and jaundice is not frequently seen. Rather, most patients present with other conditions such as cholecystitis, cholangitis, or pancreatitis. Ultrasonography or endoscopic retrograde cholangiopancreatography (ERCP) is helpful in demonstrating cysts. Nonsurgical treatment of these cysts results in high morbidity and mortality, and therefore surgery is advised in all cases. The present recommendation is for complete resection of the cyst and Roux-en-Y choledochojejunostomy. Since malignant changes in choledochal cysts have been frequently described, complete resection rather than the performance of an internal drainage procedure is preferred whenever the resection can be done safely.