A clinical observation of the combined endometriotic lesion of the small intestine and the appendix is given below. Extragenital endometriosis is a rare pathology in which endometrioid heterotopies develop outside the reproductive system organs. At about 1825% of women suffering from the pelvic organs endometriosis, the intestines are involved in the pathological process. In this regard, it is believed that in most cases its lesion is secondary while the primary lesion of the intestine with endometriosis is rarely observed and occurs as a result of hematogenous introduction of endometrial elements into the intestinal wall. Of all parts of the intestine, endometriosis most often affects the rectum and sigmoid colon (7080%), then the jejunum, less often the cecum. The most rare gastrointestinal tract endometriosis localization is the appendix, the frequency of its lesion is 0.8%. It was carried out in a clinicopathologic analysis of 14 endometriosis cases in various parts of the intestine (4 cases of the small intestine lesions, 2 rectosigmoid part of the large intestine, 2 rectum, 2 sigmoid colon, 3 appendix, 1 combined lesion of the small intestine and the appendix). In most cases, the clinical diagnosis of extragenital endometriosis is difficult, and as a rule women come with complaints typical of acute surgical pathology: intestinal obstruction, appendicitis. An important role in differential diagnosis is given to the ultrasound examination of the pelvic organs and abdominal cavity, magnetic resonance imaging, endoscopic research methods, as well as the connection of clinical symptoms with the menstrual cycle.