Idiopathic intracranial hypertension (IIH) is a condition of increased intracranial pressure without a secondary etiology. IIH is seen frequently in young and obese women. Headache, vision problems, and pulsatile tinnitus are the most common symptoms that lead patients to physicians. IIH requires a multidisciplinary approach because it could create permanent morbidity and its treatment plan should be individualized for each patient. The aim of this review was to provide an updated overview of IIH's pathogenesis, diagnostic criteria, and treatment strategies.