Objective To explore the clinical characteristics, etiology and prognosis of orbit apex syndrome. Methods Clinical data of 18 patients with orbit syndrome was summarized and analyzed. Results All patients developed vision loss, ptosis and diplopia, such as a headache or sore eyes forehead performance. However, the onset symptoms varied including vision decline or loss in 8 cases, headache in 7 cases, eye pain in 1 case, toothache in 1 case, stuffy nose and runny nose in 1 case and exophthalmos in 1 cases. The Causes included trauma in 6 cases (33％), mass lesion in 5 cases (28％), nonspecific inflammation in 1 cases (22％), infection in 3 cases (17％). Vision and eye movements disorder were improved in 5 cases of 6 patients with trauma in which 3 mild cases recovered better and one severe case did not recovered. Of 5 patients with mass lesion, the vision and eye movement were improved in one patients with sphenoid sinus mucocele receiving surgical operation. but not in the rest 4 cases with orbital adenoid cystic carcinoma or osteosarcoma patients receiving either surgical operation or radiotherapy. Of 4 patients with nonspecific inflammatory disease, eye movements were improved in 2 mild cases but not in 2 severe cases. Of 3 infectious diseases, eye movements were improved in 1 mild case. Conclusion Orbital apex syndrome is a heterogeneous disorder with multiple etiologies, presenting with a variety of initial symptoms. The prognosis depends on causes, timely diagnosis, early diagnosis and early treatment.