A 19-year-old woman with patent ductus arteriosus and increasingly severe chest pain received various examinations including exercise stress radionuclide test, which revealed hypokinesis and decreased myocardial perfusion in the apex and left ventricular dilatation. Conventional selective coronary arteriography and newly-developed ECG synchronized digital subtraction angiography (DSA) were performed to identify the genesis of her myocardial ischemia. Selective coronary arteriography revealed congenital coronary arterial fistulae in the right and left coronary arteries. The largest fistula was observed around the obtuse marginal artery terminating in the left ventricle. Although stenosis of the left circumflex and left anterior descending arteries (LAD) were not observed, the LAD appeared to be hypoplastic. Hypoplasia of the LAD and the steal phenomenon by arterial fistula could have been responsible for the myocardial ischemia in the apex, but this method did not clearly reveal the area and the degree of myocardial ischemia in the apex. Sequentially subtracted images during the arterial and capillary phases were obtained by utilizing ECG-synchronized DSA for selective coronary arteriography. For these images, densitometric analysis was adapted to the myocardial region, i.e. circumferential densitometry using 21 segments around the left ventricular wall. Decreased myocardial perfusion was clearly demonstrated in the apex in capillary phase images. Moreover, circumferential analysis provided detailed information about the area and the degree of impaired peripheral circulation in the myocardium. This analysis by DSA may prove useful for evaluating myocardial perfusion abnormalities.