The case of a 50-year-old man with abdominal pain and abnormal liver t
est results is described. Endoscopic retrograde cholangiopancreatograp
hy with manometric studies showed biliary dilatation, poor ductal drai
nage, and sphincter of Oddi dysfunction. Clinical and radiographic clu
es suggested the possibility of an ampullary lesion; a small ampullary
adenoma was detected after endoscopic sphincterotomy, and ampullary c
arcinoma was found in the operative specimen. Malignancy can cause sph
incter dysfunction not only in the esophagus (as pseudoachalasia) but
in the ampulla of Vater as well.