Chemotherapy has few impact on neuroendocrine tumour patients outcome: it may decrease functional secretory symptoms and increase slightly median survival time. In islet-cell carcinoma of the pancreas the standard chemotherapy regimen is the combination of streptozotocin and doxorubicin. It induces 50% response rate and may increase by 50% the median survival. In enterochromaffin-cell tumours chemotherapy has modest impact and the combination of 5-fluorouracile and streptozotocin is the standard regimen. In undifferentiated enterochromaffin-cell tumours of unknown primary the standard chemotherapy regimen is the combination of etoposide and cisplatin which induces 50% response rate with probably no impact on overall survival. Chemotherapy must be indicated within the frame of a multidisciplinary approach and only in patients who have refractory and progressive disease. Indication of chemotherapy must be balanced with indications of biotherapies and embolization.