A critically ill infant with congenital complete heart block and neonatal lupus was treated with pulse steroids, exchange transfusion and intravenous gammaglobulin. Transient improvement in clinical status and laboratory results occurred, although the infant died. Based on this report and review of prior experience with immunosuppressive therapy, prenatal treatment of the mother with betamethasone or dexamethasone has been successful in resolving the cardiomyopathy/myocarditis associated with neonatal lupus. Postnatal treatment of the infant with steroids appears to be beneficial for persistent hepatic and hematologic manifestations. The evidence for exchange transfusion is less certain.