Ileosigmoid knotting (ISK), also known as compound volvulus, is a rare cause of intestinal obstruction wherein the ileum wraps around the base of the sigmoid colon and forms a knot with rapid progression to gangrene. The worldwide incidence of ISK ranges from 1 in 1500 to 1 in 66,431. ISK is particularly rare in pregnancy, with the majority of cases occurring in the third trimester. The preoperative diagnosis of ISK in pregnancy is challenging because the condition is often mistaken for other obstructive or nonobstructive emergencies and delayed because of concerns regarding imaging in pregnancy. We report a case of a gravid woman presenting at 13 weeks' gestation with features suggestive of intestinal obstruction and who underwent an emergency laparotomy during which a diagnosis of ISK was made. Her recovery was uneventful, and she was able to carry the pregnancy to term.