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In this report, a case of hemorrhagic shock caused by a gastrointestinal stromal tumor (GIST) of the ileum, which was successfully treated by emergency surgery, is presented. A 67-year-old male patient presented to the Ibaraki Medical Center, Tokyo Medical University (Ami, Japan) in July 18, 2014, with dizziness and blood in the stool. Upper endoscopy and colonoscopy failed to reveal the source of the hemorrhage, although abdominal contrast-enhanced computed tomography revealed extravasation of the contrast medium into the small intestine. The patient developed hemorrhagic shock; thus, double-balloon enteroscopy (DBE) was performed, which revealed a Meckel's diverticulum and a submucosal tumor with excessive bleeding at 60 and 100 cm proximal to the ileocecal valve, respectively. Subsequent emergency partial resection of the ileum, including the tumor and the Meckel's diverticulum, was performed in July 20, 2014. Histological examination of the excised tumor revealed proliferation of spindle-shaped cells, and immunohistochemical staining of the tumor was positive for CD34, KIT and α-smooth muscle actin, but negative for S-100 protein. These immunohistological results supported the diagnosis of GIST of the ileum. The patient had an uneventful recovery and has been monitored at our outpatient clinic for 14 months after surgery. This case demonstrated the efficacy of DBE for the diagnosis of small intestinal bleeding, and immediate emergency surgery should be considered for cases of small intestinal GISTs with excessive bleeding.