A case is reported of congenital long QT interval associated with fixed 2:1 atrioventricular block. The bradycardia was detected at 16 weeks of gestational age. The atrioventricular block was due to an extremely delayed ventricular repolarization. Early detection of bradycardia in fetal life and the demonstration of a normal positive correlation between QT duration and ventricular rate suggest that, in this case, the syndrome may be due to an anomaly of the myocardial cells rather than to imbalance of the sympathetic nervous system.