Abstract Neonatal lupus (NLE) is an autoimmune disease associated with maternal antibodies to Ro/La and characterized by cutaneous lesions, heart block, cardiomyopathy, hepatobiliary disease, and hematologic cytopenias. In most cases, only one organ is affected, although multiple organ involvement is not unusual. Since NLE is presumably caused by maternal autoantibodies, the disease process is transient. However, cardiac NLE, in particular, may be fatal or persistently disabling. Optimal therapy has not yet been determined. Mothers of babies with NLE are often initially asymptomatic, but eventually most develop symptoms of autoimmune disease, particularly diseases associated with anti-Ro/La autoantibodies, such as Sjögren's syndrome and systemic lupus erythematosus. Children who have had NLE are probably at increased risk for autoimmunity later in life, sometimes as early as pre-adolescence, but the magnitude of the risk for the children is not known. Only a small percentage of babies exposed to maternal autoantibodies to Ro and/or La develop NLE. The factors governing which babies develop disease and, if disease develops, which organs will be affected have yet to be fully elucidated. In this review the clinical features, diagnosis, therapy, and prognosis of NLE are discussed, and a summary of experimental data relating to pathogenesis is presented.