Congenital arteriovenous fistulas of the internal mammary artery are extremely rare. Here, we report a case of congenital arteriovenous fistula with an aneurysm between the right internal mammary artery and the superior vena cava of an 80-yearold woman, presenting clinically with a palpable pulsating mass over the right anterior chest wall. With the patient's consent, selective transcatheter intravascular embolization with glue (N-butyl cyanoacrylate) was performed successfully. The patient was discharged 2 days after the procedure, and had no complications at the 1-year follow-up.