Gastrointestinal stromal tumors(GISTs) are rare neoplasms of the gastrointestinal(GI) system originating from the mesenchyme. GISTs mostly develop in the stomach and small intestine. We present here a case of jejunal GIST which is the rarest subtype. A 54-year-old man presented with lower right side abdominal pain. On workup, images showed a 7cm solid-cycstic lesion adjacent to ascending colon. On surgical exploration, a large jejunal tumor en bloc resected and jejuno-jejunal primary anastomosis was performed. Pathologic results showed a 9cm jejunal GIST with 5% proliferation index. Immunohistochemistry results demonstrated high expressions of CD117, whereas CD34 negative. he patient was discharged uneventfully. GISTs should be considered in patients with abdominal pain. he mainstay treatment of the jejunal GIST is complete surgical resection. he deinitive diagnosis of GISTs is by immunohistochemical stains.