Congenital AVF (arteriovenous fistula) is a rare vascular malformation that can occur anywhere in the body. Large and medium-sized congenital AVFs are extremely rare. Since clinical manifestations are not very specific, the patient's presentation could range from asymptomatic fistula to lifethreatening congestive heart failure. Although embolization can be used as an alternative treatment for AVF, surgery is still the preferred method. A case of a 28-year-old male with a congenital AVF between the brachial artery and brachial vein presented with heart failure that was treated surgically is reported here.