Congenital abnormalities of the pancreaticobiliary tree may go undetected until adulthood. In adult patients with persistent and unexplained signs and symptoms, such as cholangitis, pancreatitis, jaundice, recurrent abdominal pain, and nausea and vomiting, a congenital anomaly of the pancreatic or bile duct must be considered and a low threshold for performing cholangiopancreatography is recommended. The most common congenital pancreaticobiliary abnormalities seen in adults are choledochal cyst, anomalous junction of the pancreatic and common bile ducts, aberrant biliary ducts, and pancreas divisum, with the following entities being more rarely seen: choledochoceles, multiple communicating intra- and extrahepatic duct cysts, Caroli disease, pancreaticobiliary abnormalities associated with situs anomalies, annular pancreas, and aberrant pancreatic ducts associated with enteric duplication cysts. Cholangiopancreatography allows confirmation of diagnosis and may demonstrate associated abnormalities such as choledocholithiasis or tumor. Recognition of congenital anomalies may aid in surgical planning and prevent inadvertent ductal injury. Although congenital pancreaticobiliary abnormalities are relatively uncommon, the increased prevalence of cholangitis, gallstones, and cholangiocarcinoma seen with the various types of biliary cystic disease and junctional anomalies and the increased association of pancreatitis seen with pancreatic anomalies make recognition of variant anatomy clinically important.