Congenital arteriovenous fistula (CAVF) is a rare cardiac lesion. Angina pectoris is uncommon in younger patients with CAVF. Fistula-related symptoms, complications of this anomaly and surgical complications have a strong correlation with the age of the patient. A ten-year-old male patient with angina pectoris in whom the diagnosis of CAVF was established, and who, following surgical ligation recovered, is presented.