Benign intracranial hypertension (BIH) is characterized by an elevation of the intracranial pressure not associated with an intracranial process or hydrocephaly, and with normal cerebrospinal fluid (CSF) contents. The elevation of the intracranial pressure is isolated; therefore, diseases such as cerebral venous thrombosis or dural fistulas should not be considered as etiologies of BIH. The exact definition of BIH remains debated, and other terms such as "pseudotumor cerebri" or "idiopathic intracranial hypertension" are often used in the literature. Although we agree that BIH is usually not a so benign disease, we suggest that BIH is still the most appropriate term to describe this entity which should be classified as "secondary BIH" or "idiopathic BIH" depending on whether there are precipitating factors for the development of BIH or not. We also propose new diagnostic criteria emphasizing the need for investigations for the diagnostic of secondary and idiopathic BIH. The management of patients with BIH depends mainly on the presence and severity of ocular symptoms and signs on which the prognostic of the disease is based. Repeated lumbar punctures associated with acetazolamide and weight loss are usually efficient enough. However a surgical treatment (optic nerve sheath fenestration or lumboperitoneal shunt) is required when appropriate medical management does not prevent progressive alteration of vision (visual loss or visual field defect), or when the patients complains of severe, refractory headaches. Careful follow-up with repeated formal visual field testing may help preventing a devastating visual loss in these patients.