A congenital coronary arteriovenous fistula is a rare condition, which is an abnormal communication of the coronary artery with the right or left ventricle, the right or left atrium or the pulmonary artery. A coronary arteriovenous fistula, complicated with a pulmonary aneurysm, is quite uncommon. A case of 68-year-old woman, complaining of resting chest pain for 1 week, is reported. The coronary arteriovenous fistula associated, with a pulmonary aneurysm, was confirmed by coronary angiography and multidetector-row helical CT (MDCT), and was surgically ligated.