A 28 year old woman was admitted to the hospital with a 4 month history of headaches and blurred vision and a 2 week progressive loss of visual acuity. On examination she was found to have complete external ophthalmoplegia, florid papilledema and an otherwise normal neurologic examination. Cerebrospinal fluid pressure and protein supported the diagnosis of pseudotumor cerebri. A normal cerebral angiogram and “slit-like” ventricles on brain computed tomography supported the diagnosis. She was treated with cortico-steroids, and acetazolamide and underwent optic nerve fenestration bilaterally. This resulted in complete resolution of the ophthalmoplegia and papilledema.