Ileal knotting is a rare but serious complication of pregnancy, with relatively high maternal and fetal mortality. We report a case of ileal knotting with complications in a pregnant woman without any history of abdominal or pelvic surgery. To our knowledge, there is no other report in the international literature. Thus, the possibility of ileal knotting should be borne in mind when investigating epigastric pain in a pregnant woman without any history of abdominal or pelvic surgery, even after the onset of labor pains. Delayed surgical intervention can result in maternal and fetal mortality.