Neonatal lupus erythematosus is a disease primarily characterized by cardiac and/or cutaneous involvement. Liver and haematological involvement occur in a few cases and these are normally considered to be mild and transient complications. The disease is suspected to be caused by transplacental passage of maternal autoantibodies, primarily anti-Ro/SSA and anti-La/SSB, from mothers with systemic lupus erythematosus. Here we report a child with neonatal lupus erythematosus who died from severe liver and haematological disease when 2 d old. The baby had no cardiac or cutaneous involvement. The mother has had two subsequent pregnancies. In the first she had a boy without certain signs of neonatal lupus erythematosus. In the next pregnancy the child developed congenital complete heart block and cutaneous lesions.