Neonatal lupus erythematosus is a rare disorder caused by transplacental autoantibodies from the mother to the fetus. This syndrome is characterized by congenital heart block and/or cutaneous lesion. A total of 10 cases of neonatal lupus erythematosus were diagnosed at the National Taiwan University Hospital from 1988 through 1998. The incidence of cardiac anomaly and other clinical features in patients with neonatal lupus erythemayosus in this study was compared with those in previous studies. Results showed that 50% of patients had congenital heart block and/or cutaneous lesion, which is compatible to previous statistics. However, the findings showed that 80% of the patients were female and 90% of the mothers had acquired an autoimmune disorder, which were much higher compared with that of other studies. To date, no definite treatment is suggested prenatally to prevent the occurrence of congenital heart block, but careful maternal screening and serial fetal echocardiogram are warranted.