Idiopathic intracranial hypertension (IIH) is a disease of unknown aetiology. IIH typically affects young obese females. The majority of the cases are complicated by papilloedema, visual loss and significant headache. Weight loss is, in addition to medical therapy, the mainstay of IIH management. Medical therapy is currently solely based on clinical experience. Malignant cases with rapid visual deterioration may require immediate surgical treatment in order to prevent irreversible visual loss. Early identification is essential, and increased awareness of IIH is important.