Neonatal lupus erythematosus, characterized mainly by congenital heart block and transient skin lesions, is usually self-limited. A patient with history of neonatal lupus erythematosus and congenital heart block developed central nervous system vasculopathy resembling moyamoya disease and hypertension at 17 years of age. Careful examination and follow-up of possible central nervous system complications later in life might be important in infants with neonatal lupus erythematosus.