Combination of the primary congenital coronary artery anomalies, fistula and atherosclerosis rarely occur. We report a 50-year-old male who presented with severe chest pain, for which he underwent a coronary angiography. Injection into the right coronary artery (RCA) demonstrated a double RCA running very closely together in the atrioventricular groove. The superior one gave rise to a coronary fistula leading to the right ventricle and the inferior one was atherosclerotic. Both of them terminated as a posterior descending artery. The left system showed occlusion of the left anterior descending and major obtuse marginal. Both were corrected surgically.