Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, can be a serious vision-threatening disease. Visual acuity, visual fields, and ocular fundus appearance should be followed closely in all patients with IIH. Obese patients with IIH should be encouraged to lose weight. Medications that might cause or exacerbate IIH should be identified and discontinued if possible. Mild headaches can be treated with nonsteroidal anti-inflammatory drugs (NSAIDs) or migraine prophylactic agents. Some patients may not require additional treatment if they are otherwise asymptomatic and have no evidence of vision loss. Symptomatic patients (significant headache, visual complaints, tinnitus) or patients with visual field or acuity loss should be treated initially with acetazolamide. Furosemide may be a useful second-line agent. If vision loss is progressive despite maximal medical therapy or severe at the time of diagnosis, surgical intervention may be required. Optic nerve sheath fenestration is effective and safe, and may be repeated if initially unsuccessful. Lumboperitoneal shunting is also an option, especially if symptoms of headache are prominent and refractory to medical therapy, but it has significant complication and failure rates. Bariatric surgery can be an effective treatment for IIH in severely obese patients, but is not a useful acute intervention. Special issues must be considered when treating IIH in children or pregnant women.