Although endometriosis involves the small intestine only rarely, when present, this condition may simulate a variety of inflammatory and sometimes neoplastic conditions because of its nonspecific symptoms. We are reporting here, a case of ileal, caecal and appendiceal endometriosis which presented in the emergency as a case of an acute intestinal obstruction with a long history of symptoms which were referable to the GI tract. The patient had the diffuse involvement of a segment of the ileum with mural thickening and luminal narrowing, which led to obstruction. The literature on small bowel endometriosis has been reviewed and the clinical and pathological features have been discussed.