Abstract: Optic nerve sheath meningioma (ONSM) is a rare and slow-growing benign tumor that arises from arachnoid epithelial cells. ONSM primarily affects middle-aged women, and its clinical manifestations are mainly painless-progressive vision loss. Diagnosis is largely based on the combination of clinical and imaging findings. The typical CT/MRI findings are tubular or fusiform thickening of the optic nerve, followed by a ” double-track sign” after enhanced scan, which also may be accompanied by calcification. Treatment includes observation, radiotherapy and surgery, among which radiotherapy is the first choice for the treatment of ONSM. At present, there are many radiotherapy methods for ONSM, including spatially fractionated radiation therapy (SFRT), intensity modulated radiation therapy (IMRT), stereotactic radiosurgery (SRS), proton beam radiotherapy (PBT), 3-dimensional conformal radiation therapy (3D-CRT), etc. Surgical treatment can cause vision loss in patients and is usually not be used, but new surgical approaches have been proposed in the literature to prevent tumor extension to the contralateral side. Drug therapy has yielded vision improvement in some cases and is expected to be a potential treatment for ONSM. The mortality of ONSM is almost zero, but the visual prognosis is generally poor.(Int Rev Ophthalmol, 2023, 47: 378-383)

Key words: optic nerve sheath meningioma, diagnosis, treatment