We describe a case of a young patient with multiple occlusions of major cerebral arteries and Sjögren's syndrome. This 17-year-old female patient experienced repeated transient ischemic attacks of right hemiparesis, speech disturbance, and unconsciousness. Angiography revealed progressive occlusion of the bilateral carotid and vertebral arteries. Examinations, including a serological test, a rose bengal test, Ga scintigraphy, and a biopsy of the parotid gland, indicated Sjögren's syndrome. The patient was successfully managed with bypass surgery. Patients with Sjögren's syndrome may experience progressive occlusion of the major cerebral arteries resembling that of moyamoya disease.