Ileosigmoid knotting (ISK) is a rare type of intestinal obstruction caused by knotting of the mesentery of the ileum or sigmoid colon that rapidly progresses to gangrene with a high risk of mortality and high morbidity. The incidence of ISK is not well established, but it is higher in regions with high rates of sigmoid volvulus and in countries along the sigmoid belt. Clinical presentation is that of both small-bowel and large bowel obstruction and includes vomiting and nausea, abdominal pain, tenderness, and distention, with constipation. A contrast-enhanced computer tomography (CT) scan is the preferred modality for imaging. Management involves hemodynamic stabilisation with correction of shock using aggressive fluid resuscitation, electrolyte balance and commencement of antibiotics. Principles of surgery include resection of the knot, resection of the gangrenous bowel and establishing intestinal continuity. The outcome is generally complicated by peritonitis and sepsis that lead to mortality.