We sought to characterize the clinical nature of atrial flutter (AFL) in a large cohort of infants.There are no large studies describing the natural history of AFL in infants. Previous studies vary in the therapy used and expected prognosis.We reviewed the records of all children younger than 1 year of age who were diagnosed with AFL at our hospital during the past 25 years, excluding those with previous cardiac surgery.We identified 50 infants with AFL. Most, 36 (72%), presented within the first 48 h of life. Congestive heart failure was evident in 10 infants, with 6 presenting at 1 day of age, and 4 presenting beyond 1 month of age. The remainder were asymptomatic. A large atrial septal defect was the only structural heart disease. Spontaneous conversion to sinus rhythm occurred in 13 (26%) infants. Sinus rhythm was restored in 20 of 23 (87%) attempts at direct current cardioversion and 7 of 22 (32%) attempts at transesophegeal pacing; 7 required antiarrhythmic therapy. An additional arrhythmia, all supraventricular, appeared in 11 (22%) infants. The recurrence of AFL developed in 6 infants; 5 of 6 of these incidents occurred within 24 h of the first episode. All patients with recurrence had an additional arrhythmia.Infants with AFL usually present within the first 2 days of life. No association was found with structural heart disease. Direct current cardioversion appears to be most effective at establishing sinus rhythm. Chronic AFL has the potential to cause cardiovascular compromise. Atrial flutter in the absence of other arrhythmias has a low risk of recurrence. Once in sinus rhythm, infants with AFL have an excellent prognosis and may not require chronic antiarrhythmic therapy.