Abstract Background Insulinomas are rare tumours. Their clinical presentation, localization techniques and operative management were reviewed. Methods An electronic search of the Medline, Embase and Cochrane databases was undertaken for articles published between January 1966 and June 2005 on the history, presentation, clinical evaluation, use of imaging techniques for tumour localization and operative management of insulinoma. Results and conclusion Most insulinomas are intrapancreatic, benign and solitary. Biochemical diagnosis is obtained during a supervised 72-h fast. Non-invasive preoperative imaging techniques to localize lesions continue to evolve. Intraoperative ultrasonography can be combined with other preoperative imaging modalities to improve tumour detection. Surgical resection is the treatment of choice. In the absence of preoperative localization and intraoperative detection of an insulinoma, blind pancreatic resection is not recommended.