Primary endocrine neoplasms of the biliary tract are exceedingly rare. We r
eport on a 60-year-old man with diarrhea, vomiting and gastroesophageal ref
lux disease, with a primary gastrinoma of the common hepatic duct. The tumo
r was positive for a variety of immunohistochemical markers. Postoperativel
y the patient's symptoms disappeared and in the follow-up the patient was s
ymptom free. To our knowledge, this is the first case in the literature of
a primary gastrinoma in the common hepatic duct. The fact that the common h
epatic duct is not located within the gastrinoma triangle made the diagnosi
s difficult and the distinct localization made the surgical treatment deman
ding.