A 67-year-old man presented with leg edema. Laboratory data showed elevated blood glucose and carbohydrate antigen (CA) 19-9 levels, and anemia. Further imaging studies revealed a relatively clear-margined tumor totally occupying the main pancreatic duct (MPD) from the head to the tail of the pancreas (maximum diameter 10 cm) without mucin hypersecretion. Total pancreatectomy with splenectomy and regional lymphadenectomy were performed. Intraductal tubular carcinoma (ITC) was diagnosed by immunohistochemical staining and electron microscopic examination. Previous reports showed that this tumor is characterized by slow growth, with a favorable prognosis and intraductal nodular growth occupying the MPD and no macroscopic mucus. Whether ITC should be distinguished from other types of pancreatic neoplasm is controversial, and the accumulation of more ITC cases and multi-institutional analysis are necessary to establish the diagnostic criteria and characteristics of this histological entity.