In a 50-year-old man with extrahepatic cholestasis and iron-deficiency anaemia, local operative excision of a 4 cm submucous tumour of the papilla of Vater revealed on histological and immunochemical examination a somatostatinoma. Subsequent duodenopancreatectomy further demonstrated in the surgical specimen a lymph node metastasis and a 5 mm somatostatinoma in the papilla minor. The patient has now been free of symptoms for 12 months on substitution treatment with pancreatic enzymes.