Neonatal lupus erythematosus (NLE) is a rare variety of lupus, which occurs in 1-2% of children born to mothers with SLE, Sjögren’s syndrome, undifferentiated connective tissue disease or asymptomatic, positive for Ro/La antibodies. The most common manifestation of NLE is skin involvement and third-degree congenital heart block. Hepatobiliary disease and/or haematological abnormalities can also be seen. In the sera of neonates maternal Ro/La antibodies are present. Skin changes are similar to subacute cutaneous lupus erythematosus in adults and frequently are provoked by UV exposure. Third-degree heart block usually begins during the second trimester of gestation and is irreversible. The other symptoms tend to resolve completely at the time of serological negativization. In the article pathogenesis of cutaneous and cardiac changes as well as management during high-risk pregnancy is discussed.