Controversies in the therapy of congenital complete heart block are reviewed in terms of the timing of pacemaker implantation, the type and complications of pacing and its role in the presence of myocardial dysfunction. Drug treatment may be useful in selected cases in the presence of pleural effusions, ascites and hydrops of the fetus, but have no effect on complete heart block. Administration of fluorinated steroids in anti-Ro antibody-positive mothers with the aim of preventing complete heart block has given controversial results. Because of the variety of the clinical presentations, especially in regard to pacing therapy, it is mandatory to refer patients with congenital complete heart block to specialized centers with adequate resources and experienced personnel.