BACKGROUND Gastrointestinal stromal tumor (GIST) is a rare mesenchymal tumor identified by a positive immunostaining (KIT tyrosine kinase). Surgical resection is the primary treatment, but non-resectable and recurrent disease may be treated by specific tyrosine kinase inhibitors. This study evaluates the clinical and pathological presentation of a Danish GIST population. METHODS Retrospective evaluation of patients diagnosed, treated and/or followed under the GIST diagnosis at the Department of Surgery, Odense University Hospital, during the period from January 1995 to September 2006. RESULTS 41 patients with GIST were identified (25 males, 16 females, median age 62 years). The predominant symptoms leading to the diagnosis were upper gastrointestinal bleeding and abdominal pain. The majority of tumors were located in the stomach (63%) or in the small intestine (22%). 95% of the patients had a complete surgical resection, whereas resection was impossible in 2 patients. Approximately (2)/3 of the patients had a low or medium risk of developing metastases according to tumor size and number of mitoses. 1 in 4 patients was treated with a tyrosine kinase inhibitor during the course of the disease. CONCLUSIONS GIST is a rare mesenchymal tumor but due to immunostaining methods the detection rate is increasing. The clinical and pathological presentation of this Danish GIST population is comparable to international data.