Sinus of Valsalva aneurysm (SVA) is an infrequent cardiac anomaly. Variations in the origin and course or distribution of the epicardial coronary arteries are rarely found in the population. SVA can be acquired, secondary to infectious, degenerative or traumatic processes. This paper describes congenital right SVA and abnormal origin of conus branch of right coronary artery as a cause of acute coronary syndrome. After surgical repair of the SVA, the prognosis is usually good, and the risk of recurrence is rare.