Since the description of the Zollinger-Ellison syndrome in two patients in 1955, there have been significant advances in the understanding of its pathogenesis, natural history, relationship to multiple endocrine neoplasia type 1, diagnosis, methods of tumour localization and management. The main focus in treatment is now shifting from management of the gastric acid hypersecretory state which can now be controlled medically in almost every patient, to the management of the gastrinoma. Recent studies are beginning to provide insights into the natural history of gastrinomas, factors that are associated with invasiveness in some gastrinomas, defining the role of surgery in managing patients with different disease extents, or with MEN 1 and being able to provide insights into molecular abnormalities that may be important in their pathogenesis. In this article each of these advances is briefly reviewed with emphasis primarily on recent advances.