A general characteristic of GEP endocrine tumours is that vast majority produce and secrete a multitude of peptide hormones and amines. The rarity of these types of tumours, their possible episodic expression and the variable clinical symptoms, are the reasons why patients are often diagnosed late in the advanced stages of the disease. For these reasons, the patients with advanced metastatic disease should be treated aggressively with medical and surgical therapies aimed at reducing both symptoms and complications through strategies that reduce tumour bulk and block hormonal effects. The medical treatment of functioning endocrine tumours of the gastrointestinal tract must be based on the growth properties of the tumour and includes chemotherapy, somatostatin analogs, alpha-interferon alone and associated with somatostatin analogs, chemoembolization and radiolabelled somatostatin analogs. Even if chemotherapy has been basis of therapy for these types of tumours for a long time, it is currently reserved for progressive disease and anaplastic tumours. Biotherapy, with interferon and somatostatin analogs has been demonstrated to have a significant antitumor effect and causes an improvement of symptoms in patients with functioning neuroendocrine tumours. Furthermore, these drugs produce a notable improvement in the quality of life. Radioactive targeting therapy is the most promising new treatment modality for patients who have SST receptor positive tumours.