We describe a patient with overlap syndrome in whom systemic lupus erythematosus and scleroderma were combined. The patient expired due to severe central nervous system involvernent and pneumonia. Pertinent neuropsychiatric signs of the patient included generalied seizure, impairment of recent, memory, dysarthria, mental deterioration, Balints syndrome, right sided hemiparesis and right sided central type facial weakness. Hrain MRI and cerebral angiogram slowed cerebral vasculopathy. We suggest that a long-term follow-up is necessary for the confirmation of ciagnosis of a connective tissue disease, because the evolution, transition and overlapping features among the group of connective tissue diseases may commonly occur over time. (Kor J Dermatol 1994; 32(2): 347-351)