The case of a patient who had a cephalic duodenopancreatectomy performed because of a nonfunctional neuroendocrine tumour located in the pancreatic head is reported. Such tumour was diagnosed by a CT scan, an echoendoscopy and some punctures of the tumour to take some samples for cytology. The final anatomopathological study informed about the presence of an ampullary GIST tumour, which is a very infrequent entity, so a bibliographical search and a revision of the cases published up to this day was done.