A case of optic neuropathy and central nervous system disease developed in association with primary Sjögren's syndrome is described. Initial visual loss was partially reversible with intra veinous steroid therapy but four months later she underwent a controlateral visual loss and showed for the first time clinical manifestation of sicca syndrome. Fluorescein angiography showed signs of ischemic choroidopathy. Laboratory studies and histological findings on minor salivary gland biopsy was suggestive of primary Sjögren's syndrome. Attempts to taper oral steroid therapy resulted in worsening neurological disease and immunosuppressive therapy by azathioprine was successfully added to her regimen. Because of therapeutics and prognosis consequences, primary Sjögren's syndrome should be considered in differential diagnosis of optic neuropathy.