This review summarizes the epidemiology, etiology, clinical manifestation, treatment, follow-up of neonatal lupus erythematosus with focus on new discoveries on the etiology of the disease in recent years including anti-SSA/Ro and anti-SSB/La antibodies, serotonin (5-hydroxytryptamine 4), apoptosis of cardiac cells, calcium channels, maternal micro-chimera, genetic variants, to improve clinician awareness of the disease.