Neuropsychiatric symptoms are recognized to occur in a significant percentage of systemic lupus erythematosus patients and to be a leading cause of morbidity and mortality in lupus. Recent findings regarding the clinical presentation, diagnosis, pathogenesis, and treatment of neuropsychiatric lupus erythematosus are reviewed. The study of neurocognitive deficits and psychosocial functioning in systemic lupus erythematosus patients continues to be an area of great research interest worldwide. Severe neuropsychiatric manifestations can be divided into diffuse, focal, and seizure presentations, which can each have a different etiopathogenesis. New techniques for magnetic resonance imaging and single-photon emission CT of the brain may improve the utility and sensitivity of these neuroradiographic tests. Certain combinations of serologic, cerebrospinal fluid, and neuroimaging tests appear to be most useful diagnostically when ordered based on the patient's neurologic presentation. The role of complement, cytokines, and endothelial cell activation in causing the vascular pathology observed in the brains of neuropsychiatric lupus erythematosus patients is an area of promising research. Treatment remains empiric, but intravenous pulse cyclophosphamide and intrathecal administration of immunosuppressive medications are new approaches that have been used successfully to treat patients with severe and refractory symptoms.