Pseudotumor cerebri is an intracranial increase of CSF pressure which can occur either as an idiopathic or as a symptomatic type. The diagnosis is made according to the criteria of the International Headache Society. The pathogenesis of the idiopathic type is poorly understood. Risk factors are female sex and obesity. Symptomatic types can be caused by intoxications or different types of hydrocephalus. The treatment of symptomatic types is primarily the treatment of the underlying disorder. Furthermore, weight reduction and drug treatment with acetazolamide, topiramate, or furosemide are helpful. Surgical procedures are possible but should be used only if all other possibilities have not been successful.