Gastrointestinal Stromal Tumors (GISTs) are rare tumors of the digestive tract. The small intestine is the second most frequent localization of GIST after the stomach. We report a case of the ileal stromal tumor. The patient was admitted with a picture of hemorrhagic shock due to massive rectal bleeding. The fiberscope performed after stabilization was not objectified at the origin of the document while the CT scan objectified a mass of tissue density at the level of the right iliac fossa. Given the increasing evolution with the recurrence of patients to a target of a laparotomy for diagnostic and therapeutic purposes objectifying the tumor of the right iliac fossa at the expense of the ileum. The surgical procedure consisted of a major segmental resection of the tumor with ileo-ileal anastomosis. The anatomopathological study confirmed the diagnosis of the stromal tumor with good evolution. Through a general review of the literature, we have described the various clinical, para-clinical, anatomopathological, prognostic, and current therapeutic aspects specific to these tumors.