Medical treatment of idiopathic intracranial hypertension is often ineffective. Cerebrospinal fluid diversion or optic nerve sheath fenestration is employed when medical therapy fails. Here, we describe a case of a 13-year-old girl with idiopathic intracranial hypertension refractory to long-term trials of acetazolamide and furosemide at maximally tolerated doses. After declining surgical intervention despite progression of her visual symptoms, a trial of bumetanide (0.25 mg daily) monotherapy was successful in resolution of the patient's symptoms. These results suggest bumetanide could be effective in the treatment of idiopathic intracranial hypertension, perhaps by restoring the balance between cerebrospinal fluid formation and absorption and/or by altering the volume or ionic composition of the brain's extracellular fluid compartment.