We describe 17 patients (8 girls, and 9 boys), aged 9.6 +/- 5.7 years, with paroxysmal atrioventricular block (PAVB), a condition rarely described in children. Holter monitoring documented the PAVB in 15 patients, and tilt test was performed in 4 patients (positive in 1). The electrocardiograph (ECG) was normal in 7 patients. Two patients had acquired and 11 patients had congenital heart disease (CHD). Syncope or presyncope were present in 7 patients. A normal ECG was significantly more frequent in symptomatic patients. Pauses were significantly longer in girls and in children <5 years. PAVB was recorded only during nocturnal hours in 6 patients and throughout the day in the others. The sinus rate decreased during PAVB in 6 patients and increased in 4 (generally younger girls with symptoms). Permanent pacemakers were implanted in 13 patients, including 7 asymptomatic patients with CHD and severe bradycardia. During follow-up (3.7 +/- 2.5 years), 1 patient developed complete AVB. Although PAVB was still present in 91% of paced patients, symptoms did not recur because pacing prevented the pauses. In conclusion, PAVB is a rare arrhythmia. Autonomic nervous system dysfunction seems to play an etiological role and permanent pacing was an effective treatment.