The prognosis of neuroendocrine tumours of the gastrointestinal tract and pancreas is difficult to estimate due to their heterogeneous nature. Survival without dissemination may reach 90%, while metastases reduce the 5-year survival to less than 50%. Radical surgery offers the only possibility of cure. Palliative therapy includes surgery and intra-arterial embolization of hepatic metastases, chemotherapy, biotherapy with interferon-alpha and radionuclear treatment. Tumour-targeted therapy with tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors or monoclonal antibodies are under investigation in phase-III studies.