Neuropsychiatric syndromes in systemic lupus erythematosus (SLE) are one of the many clinical manifestations in which this pathology presents. They have a wide range of prevalence, from 37- 95% due to factors like absence of standardized definitions and non-nespecific clinical manifestations. Physiopathology is mediated by autoimmune mechanisms commonly differentiated in ischemic and inflammatory; there is a clear relationship between the pathologic pathway and the neuropsychiatric manifestation. Moreover, the blood-brain barrier plays a key role, since an alteration of the permeability allows the pass of autoantibodies to the cerebrospinal fluid. There are 19 neuropsychiatric syndromes described which include both diffuse and focal manifestations. The diagnosis must be of exclusion in sights of the more prevalent, severe and potentially deadly etiologies of the neuropsychiatric manifestations, being indispensable to conduct a full study of the patient. The therapyfocuses on symptomatic treatment for each manifestation. Immunotherapy and antithrombotic treatments should be prescripted depending on the underlying pathophysiological mechanism; however, to uncover the predominant pathological route remains a challenge. Future studies should be focused in a better understanding of the physiopathological routes in order to develop standardized diagnosis criteria and optimize an early treatment. This would have a major impact in the life of patients suffering from neuropsychiatric manifestations of SLE, whose late diagnosis is linked with greater organic damage and a poorer quality of life.