Neuropsychiatric lupus (NPSLE) manifestations are common in adults and children and are associated with an increase in both morbidity and mortality. Cognitive dysfunction, when standardly assessed using sensitive neurocognitive instruments, is the most common NPSLE manifestation. The pathogenic etiologies of NPSLE manifestations are likely to be multifactorial and may involve autoantibody production, microangiopathy, intrathecal production of proinflammatory cytokines and athersclerosis. It is becoming more clear that the integrity of the blood-brain-barrier is very important in SLE-related neuropathology. Brain imaging is an important tool that allows us to evaluate the living brain. Thus far, anatomic brain imaging has revealed abnormalities such as subcortical white matter lesions and cerebral atrophy, but these findings are non-specific. Methods that evaluate metabolic processes and other functional imaging techniques have more promise as surrogates for central nervous system damage. This article reviews the current literature on clinical and imaging aspects of NPSLE.