Abstract
The classical clinical presentation of optic nerve sheath meningiomas is painless, slowly progressive, unilateral optic neuropathy with a swollen and/or pale optic disc. It affects middle-aged women more frequently than men. Optic nerve sheath meningiomas occur frequently in patients with neurofibromatosis type 2. This association parallels the well known link between neurofibromatosis type 1 and optic gliomas. Due to advances in neuroimaging, management decisions have to be made in patients with excellent visual function. Fractionated conformal stereotactic radiotherapy holds the best potential to improve, prevent or at least delay severe visual loss in patients with optic nerve sheath meningiomas.