Endocrine pancreatic tumours (EPTs) are uncommon, with a major challenge to alert physicians to their recognition and requirements of treatment. Functioning EPTs cause well-known clinical syndromes of hormone excess. Insulinomas, gastrinomas and glucagonomas are most common; vipomas and somatostatinomas are rare. EPTs also occur as non-functioning lesions without symptoms of hormone excess occasionally with ectopic hormone, such as ACTH and Cushing syndrome as a late complication. The majority of EPTs are sporadic, but they may also be part of a multiple endocrine neoplasia type 1 syndrome or rarely the von Hippel-Lindau syndrome. EPTs have been of great interest to endocrine surgeons and we have, during recent years, witnessed continuing advances in diagnosis, imaging and treatment of the different tumour entities. It has become obvious that surgical treatment of these tumours is of increased concern and can have a marked impact on symptoms and survival.