A case of a 66-year-old patient with a history of paroxysmal supraventricular tachycardia, hypertension and chronic obturatory pulmonary disease is presented. The patient was admitted to the hospital due to acute myocardial infarction. Coronary angiography revealed a single coronary artery originating from the right sinus of Valsalva without any significant lesions. Literature concerning this rare coronary anomaly is discussed.