Sj-gren’s syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytes infiltration in the exocrine glands. Central nervous system complications of primary SS are not rare, but ischemic stroke has been rarely reported. Here we report a 43-year-old female with a two-year history of primary SS, presenting with sudden cerebral infarction. Her primary SS was diagnosed on the basis of clinical features, high levels of serum anti-SSA and anti-SSB antibodies, salivary gland secretion evaluation and positive sublingual gland biopsy results. Magnetic resonance imaging revealed infarct lesions in the parietal and occipital lobes, as well as in the left basal ganglia. Magnetic resonance angiography showed a remarkable stenosis in the left middle cerebral artery. Other differential diagnoses were ruled out. Corticosteroid and immunosuppressor, together with anti-platelet and statin were effective, and the patient recovered quickly without sequelae. Based on these findings, vasculitis due to primary SS should be considered among the causes of stroke. The literature was reviewed and the relationship between primary SS and cerebral infarction explored. The pathogenesis of ischemic stroke in primary SS is still unknown and warrants further studies.