Intestinal obstruction is a common cause of emergency surgical admission. The most frequent causes are well known and may often be safely treated conservatively in the first instance. The rarer causes of intestinal obstruction require prompt diagnosis and surgery if they are not to progress rapidly to strangulation and gangrene. One such cause is the ileosigmoid knotting, which is associated with a high morbidity and mortality. With increasing travel activity and population migration this condition is now being seen outside its original geographical sites of origin. This article focuses on the aetiology, pathophysiology, clinical features, investigations and the various surgical options for the management of the ileosigmoid knotting. Studies and case reports in English literature were identified by PubMed, ISIS, Embase and CAS searches between the years 1966–2004 using the following free text keywords: ileo- sigmoid knotting, ileosigmoid knot(ting), intestinal knot(ting), compound volvulus and double volvulus. All the reference lists were reviewed to retrieve additional articles. Aggressive resuscitation, prompt surgical relief of obstruction, appropriate antibiotics, accurate intra-operative assessment of the viability of the involved loops of intestine and the use of modern postoperative intensive care will help reduce the mortality and morbidity associated with this life threatening condition.