Giant single left coronary artery with right ventricular fistula diagnosed in early childhood.

We present an unusual case of an 18-month-old boy, who presented with dyspnea and recurrent respiratory tract infections. Echocardiography and subsequent angiography were suggestive of a fistula from a coronary artery to the right ventricle. Finally, only on table could the actual diagnosis of a single left coronary artery with right ventricular fistula be made. Surgical treatment was successfully performed with off-pump ligation and the patient was well with no residual fistula on echocardiogram when seen at 3 month follow-up.