Idiopathic intracranial hypertension is common in obese women and can lead to significant visual impairment. First described more than 100 years ago, the cause of the disorder remains unknown. Despite a multitude of proposed links, the aetiology has never been established. Impairment of cerebrospinal-fluid reabsorption is the most likely underlying pathophysiological cause of the raised pressure, but this notion has yet to be proven. Cerebral venous sinus abnormalities associated with the disorder need further exploration. Although the major symptoms of headache and visual disturbance are well documented, most data for disease outcome have been from small retrospective case series. No randomised controlled trials of treatment have been done and the management is controversial. The importance of weight loss needs clarification, the role of diuretics is uncertain, and which surgical intervention is the most effective and safe is unknown. Prospective trials to examine these issues are urgently needed.