Neuroendocrine neoplasias of the gastrointestinal tract belong to rare tumours, despite the dramatic incidence and prevalence increase in last decades. It goes together with better diagnostic and treatment modalities. When carcinoid syndrome is present, suspicion for this disease is very high. In other cases the symptomatology is nonspecific. Localisation of the primary tumour is often difficult. In the therapy surgical resection and debulking therapy dominate. The medicamentous treatment includes somatostatin analogues, biological treatment with everolimus, in high grade tumours chemotherapy, in some cases also radionuclide treatment.