Neurological involvement in the course of Systemic Lupus Erythematosus (SLE) is a challenging for the large number of patients it affects. It has a profound impact on morbidity, mortality, and quality of life. This review provides an update on current concepts regarding pathogenesis, clini-cal presentation, diagnosis issues, and management strategies for neurological SLE. The patho-physiology is complex and involves immune-mediated and vascular mechanisms of neurological involvement in SLE, including autoantibodies, neuroinflammation, dysregulation of complement, and genetic predisposition. Neuropsychiatric systemic lupus erythematosus (NPSLE) can present with a broad spectrum of central and peripheral nervous system manifestations, ranging from neuropsychiatric SLE, one of the most challenging manifestations, such as cognitive dysfunction, mood disorders, and psychosis, to cerebrovascular disease with an increased incidence of stroke and transient ischemic attacks; demyelinating syndromes mimicking multiple sclerosis; and neuropathies of sensory, motor, or autonomic function. The diagnosis of neurological SLE is very challenging due to subtle, nonspecific, and fluctuating symptoms. A meticulous neurological ex-amination along with neuroimaging techniques, autoantibody profiling, and cerebrospinal fluid analysis is needed. Current treatment strategies will be discussed with corticosteroids, conven-tional immunosuppressants, and emerging biological agents targeting specific immune pathways. It is worth mentioning the complexities of neuropsychiatric symptom management, seizure man-agement, and neuropathic pain.