Introduction: Neuropsychiatric systemic lupus erythematosus is often clinically challenging to diagnose, treat and monitor. Although brain magnetic resonance imaging is frequently performed before lumbar puncture in neuropsychiatric systemic lupus erythematosus, it is not clear from the literature whether specific brain magnetic resonance imaging findings are associated with distinct clinical features of neuropsychiatric systemic lupus erythematosus. Methods: We conducted a systematic review and meta-analysis on published studies of neuropsychiatric systemic lupus erythematosus including brain magnetic resonance imaging and the 1999 American College of Rheumatology-defined clinical neuropsychiatric systemic lupus erythematosus syndromes to determine their relationship. Pooled prevalence and risk ratio for distinct neuropsychiatric systemic lupus erythematosus associations were determined with 95% confidence intervals. Results: Of 821 studies screened, 21 fulfilled inclusion criteria. A total of 818 participants were evaluated (91% female) with 1064 neuropsychiatric systemic lupus erythematosus episodes assessed. Neuropsychiatric systemic lupus erythematosus features included headache (24%), seizures (19%), cerebrovascular disease (18%), cognitive dysfunction (15%) and acute confusional state (14%). Normal magnetic resonance imaging was significant for anxiety disorder (risk ratio: 9.00; 95% confidence interval: 2.40, 33.79), autonomic disorder (risk ratio: 7.00; 95% confidence interval: 0.51, 96.06) and plexopathy (risk ratio: 5.00; 95% confidence interval: 0.81, 31.00). Highest risk ratio of neuropsychiatric systemic lupus erythematosus syndrome with abnormal magnetic resonance imaging was observed for cerebrovascular disease (risk ratio: 0.15; 95% confidence interval: 0.10, 0.24) and demyelination (risk ratio: 0.11; 95% confidence interval: 0.02, 0.72). Conclusion: Normal magnetic resonance imaging in neuropsychiatric systemic lupus erythematosus was the most significant correlate from our meta-analysis for psychological symptoms including anxiety and peripheral nerve features of autonomic disorder and plexopathy. The main abnormal brain magnetic resonance imaging correlates included cerebrovascular disease and demyelination. Brain magnetic resonance imaging correlates poorly with neuropsychiatric systemic lupus erythematosus features, and specific clinical symptoms should be the main determinants of performing magnetic resonance imaging rather than presence of neuropsychiatric systemic lupus erythematosus per se.