The authors report on a case of voluminous perforated gastrointestinal stromal tumor (GIST) of small intestine and make a review to the light of most recent clinicopathologic advancements. The first clinical manifestation as acute abdomen due to their perforation extremely rare. Gastrointestinal stromal tumor (GIST) represent a rare group of multiform tumors with various biological behaviour. Were identified in the past as leiomyomas, leiomyosarcomas or leiomyoblastomas, has been reclassified on immunochemical features, with a positive expression of Kit (CD117 antigen). Traditionally the prognostic factors of these tumour are: mitotic rate, tumor size and anatomic site. The complete surgical resection and use of imatinib mesylate (Gleevec) are the best available approach.