The presenting features of periampullary cancer have been reviewed. Unless the neoplasm is discovered as an incidental finding at surgery, diagnosis is not made until the onset of symptoms (pain, jaundice, weight loss), which is not early, even though these tumors are close to the bile duct or ampulla of Vater. Individual philosophies aside, the prognosis for ampullary, duodenal, or distal bile duct tumors reflects the grade and stage of tumor as much as the operation done. Pancreatoduodenal resection, standard or modified, should be done with an attempt to cure and not to palliate. Statistics that show superior results after resection compared with palliative procedures reflect earlier stages lesions, healthier patients, and, most importantly, patient selection.