Introduction: Gastrointestinal stromal tumors constitute a distinct group of rare gastrointestinal tract tumors that originate from the interstitial cells of Cajal. These jejunoileal lesions are a rare cause of obstruction but can be associated with substantial morbidity.Case: A 59-year-old woman presented to the emergency department with abdominal pain and distention. Physical examination revealed tenderness and rebound in right lower quadrant. Computed tomography revealed a mass in lower right quadrant. A 9x9x4 cm exophytic ileal mass was observed at exploration. Preoperative diagnose was a small bowel tumor and then segmental resection and primary anastomosis were performed. Histopathological investigation revealed spindle cells that stained strongly for C-117, consistent with a diagnosis of a malign gastrointestinal stromal tumor.Conclusion: We conclude that exophytic small bowel gastrointestinal stromal tumors are rare lesions, which should be kept in mind by physicians among the diagnosis of small bowel obstructions in order to reduce substantial morbidity and mortality.