Abstract Background: Unroofing of anomalous coronary artery originating from the opposite sinus of Valsalva has become the procedure of choice for this congenital lesion, with surgery performed in children as young as two years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication whether surgical repair should be done in this age group. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants. Methods: Between April 2002 and February 2007, eight patients underwent surgical repair of anomalous coronary artery arising from the opposite sinus of Valsalva and coursing between the aorta and pulmonary artery. Patients’ age varied from two months to 28 years with a mean of 11.7 ± 11.1 years. Surgical Technique: Surgical repair involved unroofing the intramural segment of the anomalous coronary artery using cardiopulmonary bypass. Results: Two patients were younger than one year (Group A), and six patients were older than one year (group B). The mean intensive care unit stay was 2.5 ± 0.7 days for Group A and 2.8 ± 1.9 for Group B. The mean hospital stay was 4 ± 1.4 days for Group A and 4.3 ± 2.4 days for Group B. There was no mortality and no complications. The mean follow-up period is 14 ± 15.7 months with a range of one to 39 months. At the time of the last follow-up, all patients were asymptomatic in New York Heart Association class I and follow-up echocardiography on six of eight patients showed wide open coronary ostium. Conclusion: Unroofing the anomalous coronary artery arising from the opposite sinus of valsalva can be done in infants with minimal morbidity and mortality. Longer follow-up is needed to assess long-term results.