A 42-year-old woman developed right-sided hemiparesis due to left-sided encephalomalacia revealed by CT scan. Subsequent angiography revealed vasculitis of several intra-cranial arteries. The ESR was 65 mm/h. Further laboratory tests revealed no evidence of systemic disease so that no causal diagnosis could be posed. Treatment with prednisone (3 X 30 mg daily) led to complete cure of the hemiparesis within 6 weeks. Sixteen months later, the patient developed cutaneous lesions in the neck. Histological examination of these lesions indicated the presence of systemic lupus erythematosus (SLE). Neurological presentation of SLE is exceptional, while cerebral vasculitis as initial symptom of SLE has never been described before.