Objective To detect the clinical manifestations, diagnos is and treatment of painful ophthalmoplegia syndrome. Methods The data of onset, clinical m anifestations, laboratory examination, imaging and treatment from 12 patients with painful ophthalmoplegia, hospitalized from Mar, 2000 to Aug. 2005, were retro spectively analyzed. Results Multiple characters and extents of the headache were found in these 12 patients. The involved cranial nerves included the Ⅲ,Ⅳ, V1-2 and Ⅵ, especially the cranial nerve Ⅲ(83.3%). Several simultaneously in volved cranial nerves were frequently found (75%). Diseases which could cause hea dache along with ophthalmoplegia must be excluded before the diagnosis of the painful ophthalmoplegia syndrome was established. The examination of imaging was important for the diagnosis of painful ophthalmoplegia syndrome. Patients were sensitively responsive to cortico-steoid therapy. The cure rate was 75%. Conclusion The features of clinical manifestations, imaging results and the patients response to cortico-steoid therapy accorded with the etiology of nonspecific inflammation granuloma. Cortico-steoid therapy is effective after the definitude of the disease. (Chin J Ocul Fundus Dis, 2006,22:385-386)