The goal of medical treatment in benign intracranial hypertension (BIH) is to treat intracranial hypertension symptoms as well as to preserve vision. Reducing the production rate of cerebrospinal fluid can be achieved using acetazolamide and/or furosemide (carbonic anhydrase inhibitors), although acetazolamide is the most effective drug. The use of steroids is debatable in BIH. This review focuses on the pathophysiology of these medications, followed by the report of a series of 16 pediatric patients suffering from BIH (1996-2006). BIH was idiopathic for eight children. Depletive lumbar punctures were effective, but this result was often transient. All children were treated with acetazolamide. Doses of acetazolamide (10-20mg/kg per day) must be given every 8h to respect its kinetics. This treatment has to be continued for at least several months and decreasing the dosage must be progressive. Hypokalemia is always prevented with oral potassium. There was only one true treatment failure requiring surgery. The authors therefore advise acetazolamide as a first-line treatment (combined with etiologic treatment, if available, in cases of nonidiopathic situations) in BIH.