Abstract: Orbital neurilemmoma is one of the most common orbital tumors and commonly presents as proptosis. Typical schwannoma are mainly composed of alternately distributed AntoniA and AntoniB tumor cells, most of which are AntoniA tumor cells. MRI was taken as the first choice on clinical diagnosis, and CT examination as accessory examnation, the latter mainly observing the tumor's effect on the surrounding bone mass.Histopathological examination is the gold standard for diagnosis.Attention needs to be paid to the differential diagnosis with cavernous hemangioma, malignant orbital schwannoma and so on. The most effective treatment is still surgery, and optimal treatment has not yet been found. (Int Rev Ophthalmol, 2022, 46: 283-288, cover III)

Key words: orbit, neurilemmoma