Congenital heart block is the most severe manifestation of neonatal lupus syndrome. It is a passively acquired disease where transplacental passage of maternal autoantibodies is associated with irreversible damage of the foetal cardiac conduction system. It is well established that the condition, in the absence of structural abnormalities, is strongly associated with maternal autoantibodies to the Ro/La antigens. More specifically the disease has been closely linked to antibodies to the Ro52 component of the antigen complex. Congenital heart block constitutes a unique model where specific autoantibodies target and mediate organ-specific disease. A wide panel of maternal antibodies has been discussed in literature in association with the disease and are described in this review.