Idiopathic perioptic neuritis is a term used to describe noninfectious inflammatory disorders of the optic nerve sheaths, the causes of which are unknown. In the following report, a 68-year-old woman with bilateral visual loss was found to have chronic inflammation with necrobiotic granulomas of her optic nerve sheaths. The patient, who had no systemic condition known to be associated with necrobiotic granuloma, lost vision from infarction of the optic nerve parenchyma and from compression due to thickened meninges. Although there are similarities between the inflammatory reaction in this case to the necrobiotic dermatoses, the pathogenesis of this condition remains obscure.