Central nervous system (CNS) involvement is rare in scleroderma unless there are concomitant abnormalities in renal or lung function or malignant hypertension. A 43-year-old woman with typical scleroderma developed subacute encephalopathy despite absence of the above abnormalities. Cerebral angiography demonstrated a focal arteritis. The patient improved while being given corticosteroids. We believe this case indicates that cerebral arteritis can occur in scleroderma.