The coronary sinus (CS) is the venous drainage system of the heart. CS ostium atresia is a rarely seen cardiac malformation. Congenital atresia of the CS is usually found together with persistent left superior vena cava (LSVC) and other cardiac malformations. However, isolated congenital atresia of the CS is very rare. We present a rare case of isolated congenital atresia of the CS connecting the left atrium and coronary veins without persistent LSVC in a 58-year-old female.