Control of symptoms due to hormonal secretion is an integral first-step in the management of patients with functional tumours of the pancreatico-duodenal region. Symptomatic drugs should be employed, as well as systemic chemotherapy for nonresectable tumours on the basis of histological differentiation and tumour evolution. Hepatic chemoembolization yields objective response rate of 50%. Surgery however remains the only curative option. Well-differentiated and resectable tumours should be treated by resection, associated with hepatectomy in cases of resectable metastases. Liver transplantation should be reserved for patients with well-differentiated slowly-progressive tumours demonstrating multiple hepatic metastases with a resectable primary in the absence of other metastatic disease. When medical options are inefficacious in the control of hormonal symptoms, cytoreductive surgery can be an effective option.