Intraductal tubulopapillary neoplasm (ITPN) of the pancreas has been recently reported. It is very rare, therefore clinical behavior and prognosis has not yet been characterized. We experienced a case of ITPN of the pancreas which presented with acute pancreatitis and treated with Whipple's operation. Histopathologic finding showed papillary hyperplasia with carcinomatous change. The tumor recurred after 47 month of operation, and she underwent total pancreatectomy. Pathologic finding revealed tubulopapillary growth with high grade dysplasia. Immunohistochemial staining was not performed, however gross and microscopic findings were compatible with ITPN of the pancreas. We report a case of ITPN of the pancreas.