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Background: Multi-system damage is a hallmark of systemic lupus erythematosus (SLE), a chronic systemic autoimmune disease. The typical initial symptoms of SLE are arthritis and dermatosis, whereas the presence of intracranial mass lesions as the first manifestation of systemic lupus erythematosus is very rare. This report describes an 18-year-old woman with intracranial mass lesions associated with SLE. Case Report: An 18-year-old woman was initially admitted to the hospital because of headache for 3 days, weakness in left arm, and blurred vision for 1 day. Magnetic resonance imaging (MRI) of her brain showed multiple abnormal occupying lesions in the right frontoparietal lobe. However, no evidence of tumor or infection was found. One month later, she was readmitted with right limb weakness and aphasia for 1 day. Brain MRI showed obvious and new abnormal signal shadows in both the right parietal lobe and the left frontotemporal parieto-occipital lobes compared with the previous MRI. She responded positively to immunotherapy, which, in a woman of child-bearing age, supports the diagnosis of SLE. Ultimately, the presence of focal neurological symptoms, abnormal autoantibodies (such as antinuclear antibodies, anti-dsDNA antibodies, anti-SSA autoantibodies, and anti-ribosomal P protein antibodies), as well as her positive response to immunotherapy, contributed to the diagnosis of SLE with intracranial mass lesions. No recurrence was seen during 1 year of follow-up. Conclusions: It is unusual for SLE to present with intracranial mass lesions as the initial symptoms. The pathogenesis of the neurological symptoms of the patient may be small vessel thrombosis or vasculitis leading to cerebral masslike necrosis. [ABSTRACT FROM AUTHOR]