We reported a 58-year-old woman with isolated angiitis of the central nervous system. The patient had suffered from recurrent injection in the left conjunctiva and edema in the left eyelid for three months. Head CT and MRI disclosed a left temporal mass. Carotid angiogram showed left carotid-cavernous fistula, but other vessels were in all normal shape. At biopsy, lymphocyte infiltrations and thickening in entire vessel walls, especially of veins, were found. There was no giant cell or granuloma. The left temporal mass seemed to be caused by venous infarction. Her symptoms disappeared in two weeks after admission without any medication.