Visual loss in patients with encephalotrigeminal angiomatosis or Sturge-Web
er syndrome usually results from secondary glaucoma or from damage to the r
etrogeniculate pathways by the angiomatous lesions. Optic neuropathy has no
t been reported as a component of this syndrome. This report describes a pa
tient who developed bilateral optic neuropathy with progressive visual loss
associated with diffuse cerebral angiomatosis in the setting of Sturge-Web
er syndrome.