Carcinoid tumours arise from argentaffine cells or from a primitive stem cells which may differentiate into anyone of a variety of adult endocrine-secreting cells. Carcinoid tumour of the pancreas is a very rare tumour with less than 50 cases reported in world literature. In literature it is denoted "pancreatic serotoninoma" or "serotonin-producing pancreatic tumour". Due to its rarity the tumour is an unusual cause of carcinoid syndrome. As the carcinoid tumour of the pancreas does not always causes carcinoid syndrome its absence does not necessarily exclude the existence of the tumour. The tumour is frequently malignant. Over 50% of patients have metastases at the time of surgery. This is the reason why radical surgery is not possible in a number of patients. Excisional surgery offers the best chance for recovery or long term survival. We report on a 57-year-old woman with carcinoid syndrome caused by malignant carcinoid tumour of the head of the pancreas without liver or other distant metastases; it was successfully excised with pylorus preserving cephalic duodenopancreatectomy (after Longmire-Traverso) and radical lymphadenectomy. The diagnosis was established on the basis of histologic and immunohistochemical findings. The patient is symptom free for more than eight months.