Idiopathic intracranial hypertension (IIH) typically presents with bilateral papilledema; however, highly asymmetric and rare unilateral cases have been reported. We report three cases of IIH meeting modified Dandy criteria presenting with unilateral papilledema. Magnetic resonance imaging (MRI) demonstrated bilateral distention of the optic nerve sheaths and computed tomography (CT) of the orbits demonstrated a smaller diameter of the optic canal in the unaffected eye in two cases. Papilledema fully resolved in all with acetazolamide. Of postulated mechanisms, we suspect that differences in bony optic canal diameter, compliance of the lamina cribrosa, and optic nerve sheath anatomy may contribute to asymmetry.