Congenital coronary arteriovenous fistula (CAVF) is a rare condition which is characterized by abnormal communication of the coronary artery with the right ventricle, right atrium, left atrium, left ventricle or pulmonary artery. In this paper, we report a case of a 68-year-old woman complaining of resting chest pain for one week. Initially, after performing a coronary arteriogram, the case was diagnosed as a CAVF combined with a pulmonary artery aneurysm. However, a multidetector-row CT (MDCT) was also performed, and the structure initially diagnosed as a pulmonary artery aneurysm was identified as a dilated pulmonary sinus. Subsequently, the patient was treated successfully with a simple ligation.