Congenital atrioventricular block is defined (Yater) as a documented conduction defect in a young subject with unquestionable bradycardia in the absence of a history of infection which might have caused the condition. This condition may be isolated or associated with structural cardiac disease. Isolated block is often observed in patients with mothers suffering from autoimmune disease, often clinically latent. Anti-Ro/SS-A and La/SS-B antibodies cross into the fetal circulation and cause inflammation of the fetal conduction system. The diagnosis of the conduction defect is sometimes made during fetal life by echocardiography. Echocardiography assesses fetal hemodynamic status and may detect signs of fetal deterioration. Long-term follow-up studies have revised the previously considered good prognosis of isolated congenital atrioventricular block but advances in cardiac pacing provide satisfactory treatment. Follow-up by Holter monitoring, exercise testing and echocardiography is justified in children and adolescents; the patients may become symptomatic at any age. Indications for permanent pacing in pediatric patients have been difficult to determine due to the lack of data from controlled studies and multicenter trials. Pacing is not always easy in children. Epicardial pacing is advocated in small patients (< 5 years of age or 30 kg in weight). A pacing mode which restores the normal atrioventricular sequence is theoretically superior to single ventricular pacing even with rate responsive frequency.