Objectives: We clarified the characteristics and risk factors of CVEs in young SLE patients. Method: We retrospectively reviewed the medical records of patients younger than 50 years of age diagnosed with SLE and first CVEs from 1995 to 2020 in a tertiary medical center in Taiwan. We collected data on the patient characteristics before the CVE and reviewed the laboratory data obtained during the period. At a ratio of 1:3, cases and controls were matched with sex, SLE diagnosis age, diagnosis year, and SLE duration. Results: We enrolled 43 CVE SLE patients and matched 129 non-CVE SLE controls. The median age at the time of the CVE was 39 years. Around 70% of young-aged CVE involved the cerebral lobes of frontal (similar to 30%), parietal (similar to 20%), occipital (similar to 10%), and temporal (similar to 10%). The peak incidence period for hemorrhagic CVE was within 1st year of SLE diagnosis (37%); in contrast, during the 2nd to 5th year of SLE diagnosis (25%) for ischemia CVEs. Hyperlipidemia (odds ratio [OR] = 19.36, p = 0.002), anti-phospholipid syndrome (APS) (OR = 41.9, p = 0.0068), a lower hemoglobin level (OR = 0.66, p = 0.0192), and a higher SLE Disease Activity Index (SLEDAI-2k) score (OR = 1.22, p = 0.0019) were independent risk factors for CVEs in young SLE patients. Conclusion: Hyperlipidemia, APS, low Hb level, and high SLEDAI-2k significantly increase the risk of young-aged SLE patients developing CVE. Copyright (c) 2023, Formosan Medical Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/bync-nd/4.0/).