Primary carcinoma of the ampulla of Vater is an extremely rare condition. According to Lieber,1 only one proved case of this condition has been reported, for while many cases are described in the literature, careful scrutiny of the reports reveals that a possible origin from other nearby and closely related structures has not been excluded. There are four possible sources of origin of a tumor in this region: (1) the duodenal mucous membrane covering the papilla of Vater, (2) the terminal common bile-duct, (3) the proximal main pancreatic duct, and (4) the ampulla of Vater. The lesion is seldom considered in arriving at a clinical diagnosis, and by the time the patient comes to autopsy the tumor has attained such a size as to involve two or more of the structures alluded to above. A decision as to its exact source is then obviously impossible. The case here reported is of particular interest since the patient was seen early, and the primary lesion observed at autopsy was small and self-limited so that a definite opinion could be formed as to its origin. A. P., a white man aged fifty-six years, was admitted to the Nanticoke State Hospital on Jan. 10, 1938, complaining of jaundice, epigastric pain, gaseous eructations, light stools, and dark brown urine. He had been in apparently good health until a week before admission to the hospital, when jaundice appeared insidiously and increased progressively. Two days later he experienced gradually increasing pain in the epigastric region, associated with gaseous eructations but without nausea or vomiting. His appetite was good, but, in spite of this, there had been a loss of weight of ten pounds in the last six weeks. Chills and fever were absent, and constipation was not a feature.