Gastrointestinal stromal tumours, though rare, are the most common mesenchymal neoplasms affecting the gastrointestinal tract. The most frequent sites of origin are the stomach and the small bowel, but they can occur anywhere in the gastrointestinal tract. Mesenteric and retroperitoneal forms have been described. The Authors present their personal experience with the treatment of gastrointestinal stromal tumours, with particular reference to the broad spectrum of clinical presentations and to the consequent therapeutic implications. We report on a retrospective analysis of the clinical presentations and courses, surgical management and pathological features of 27 patients with such tumours treated in our institution from 1993 to 2005. The variables analysed were the morphological and clinical characteristics of the tumours, demographic data, type of surgical treatment and postoperative course. Long-term survival was evaluated on the basis of clinical and/or telephonic follow-up in all patients. One tumour was located in the oesophagus, 14 in the gastric area, 7 in the small bowel, 2 in the colon-rectum, and 3 in the peritoneum. All patients studied received radical surgical treatment. In 7 patients surgical resection was extended to other organs. No postoperative mortality or major postoperative complications were observed. Twenty-two patients are still alive at follow-up. Three patients died as a result of neoplastic relapse and 2 of other causes. The median survival was 36 months. The actuarial 3- and 5-year survival rates were 89.7% and 67.8%, respectively. Our experience indicates that the site of origin of gastrointestinal stromal tumours with their broad spectrum of clinical presentations may influence both the therapeutic choice (neoadjuvant utilisation of imatinib mesylate) and the surgical treatment (wedge resection vs enlarged operations).