Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, with multisystemic involvement. The disease has several phenotypes, with varying clinical presentations in patients ranging from mild mucocutaneous manifestations to multiorgan and severe central nervous system involvement. Several immunopathogenic pathways play a role in the development of SLE. The lupus erythematosus (LE cell) was described by Hargraves in 1948. Several pathogenic autoantibodies have since been identified. Despite recent advances in technology and understanding of the pathological basis and risk factors for SLE, the exact pathogenesis of SLE is still not well known. Diagnosis of SLE can be challenging and while several classification criteria have been posed, their utility in the clinical setting is still a matter of debate. Management of SLE is dictated by organ system involvement, and despite several agents shown to be efficacious in the treatment of SLE, the disease still poses significant morbidity and mortality risk in patients.