Scleroderma or systemic sclerosis is a multisystem disease due to excessive collagen deposition in different organs and autoimmunity by production of autoantibodies.According to previous reports, brain is rarely affected in scleroderma, howeverrecent studies show central nervous system can be affect not only as a complication of systemic involvement (hypertension, renal failure) but also as a primary manifestation.In scleroderma, thrombus formation in central nervous system and peripheral systems is uncommonmay be due to endothelial cells damage which causes to release antithrombotic factors. We discuss a scleroderma patient with high titter of anticardiolipin antibody who developed to cerebral sinus thrombosis and cerebellum infarction. Then we review literature for both primary brain involvement and thrombotic event in systemic sclerosis.