Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure of unknown etiology. For overweight or obese patients with IIH, weight reduction of 5% to 10% of total body weight at diagnosis is a long-term treatment strategy. Though not proven, the initiation of acetazolamide can assist in symptom reduction and resolution. In patients with either fulminant IIH or those on maximal medical management with progressive vision loss, intravenous steroids and acetazolamide can be initiated while surgical options are urgently arranged. Because of its lower complication rate, I prefer to use optic nerve sheath fenestration in settings of precipitous visual decline, but I have used cerebrospinal fluid diversion surgery in settings of vision loss with severe, intractable headache. Often, the choice of surgical intervention is individualized for the patient and the available expertise. In the future, results from the ongoing multicenter, double-blind, placebo-controlled Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) will provide important data regarding the efficacy of acetazolamide and the utility of diet and exercise.