Acute limb compartment syndrome (ALCS) is a surgical emergency that can have serious consequences unless promptly diagnosed and treated, which is particularly challenging when there is an unusual cause. This is a comprehensive review of reported causes of ALCS. From 1068 included articles, we found 299 discrete causes of ALCS including toxins, infections, endocrine pathology, haematological emergencies, malignancy and iatrogenic ALCS. Familiarity with this wide range of ALCS causes may assist in early diagnosis of this limb-threatening condition.