There are a variety of tumor markers used for diagnosis of pancreatic and biliary tract cancer including carbohydrate antigens such as CA19-9, DUPAN-2, CA50 and Span-1, the carcinoembryonic antigen and pancreatic enzymes such as elastase 1. The tumor marker kinetics in serum help to predict survival in patients receiving surgical and/or non-surgical treatment. Since each of those tumor markers remains at a low sensitivity and specificity in the early stage, none are useful for detecting early cancer. In spite of the development of various diagnostic techniques, pancreas and biliary tract cancer is usually diagnosed at an incurable stage, and the 5-year survival rate is extremely low. Therefore, improvements in survival may well result from screening for early-stage pancreatic and biliary cancer. The development of tumor markers with higher sensitivity and specificity should have the highest priority.