We report a case of intraductal papillary adenocarcinoma of the distal pancreatic duct. Although a rare subgroup of exocrine pancreatic tumors, their diagnosis is an important one in view of their favorable prognosis with pancreatectomy. Because of the established behavior of villous tumors of the colon, to which they are similar histologically, these tumors should be resected, even if biopsy shows benign disease. Patients should be followed radiologically for recurrence or the development of new adenoma following resection.