Background: Cerebral vasculitis is an infrequent feature of systemic lupus erythematosus (SLE). Less than 10% of post-mortem investigations show it. Aim of the work: This study aimed to investigate the clinically manifested central nervous system (CNS) vasculitis, in relation to the clinical, laboratory, neuroimaging characteristics, as well as disease activity in SLE patients. Patients and methods: The medical records of SLE patients attending to the Rheumatology and Rehabilitation Department; Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University were reviewed. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was measured. Magnetic resonance imaging (MRI) findings for CNS manifesting patients were recorded. Results: Forty-one SLE patients with CNS manifestations and 47 SLE control without CNS affection, mean age was 29.3 ± 9.3 years, 90.8% were females. Time lapse between disease onset and diagnosis of CNS vasculitis was 5.6 ± 5.2 years. Most common CNS symptoms were convulsions and headache (29.3% each). Stroke and transverse myelitis were reported in 12.2% each. Precipitating factors prior to these manifestations either none were in remission and/or stoppage of treatment. All patients showed vasculitic findings in MRI with various brain destructive lesions; 75% had ischaemic foci (most of them were antiphospholipid negative, 72.4%) and 22% had atrophic changes. There was a statistically significant relation between SLEDAI, Anti-DNA and CNS affections (p < 0.001,0.012). Conclusion: Cerebral lupus vasculitis manifests diverse clinical CNS affection where convulsion and headache were the most common presentation. SLE patients with CNS manifestations had various changes in MRI brain highly suggestive of vasculitis and were significantly related to disease activity index.