Neuropsychiatric disease occurs in up to two thirds of all systemic lupus erythematosus patients. At present, there is no accepted classification for the myriad of neuropsychiatric manifestations that can be divided into diffuse, focal, and seizure presentations. Over the past decade, the pathogenesis of lupus cerebritis has become better understood. Consequently, the approach to the diagnosis and therapy can often be tailored to the suspected underlying etiopathogenesis. Future advancements in therapy will further improve the prognosis of patients with neuropsychiatric lupus erythematosus.