A persistent left superior vena cava draining into the left atrium associated with atresia of the coronary sinus-ostium, ASD, and PDA is a rare congenital anomaly. The patient was a 4 year-old female whose complaints were frequent URI and exertional dyspnea. The congenital heart anomaly was suspected at 2 months of her age. Chest films showed cardiomegaly [C-T ratio, 75%]. EKG, Echocardiography, cardiac catheterization and angiocardiography were performed. Open heart surgery was done under impression of LV-RA shunt, bilateral superior vena cavae, and ASD. At the time of operation, huge LA and RA, inferior vena caval defect of a secundum type ASD [1.5 x 3cm in diameter], absence of innominate vein, atresia of the coronary sinus-ostium, and persistent LSVC draining into LA were noted. Direct suture closure of ASD and ligation of LSVC were done. The patient`s postoperative course was somewhat eventful: systolic murmur at apex remained. Four months after the operation, congestive heart failure attacked a few times. PDA that was overlooked at the time of open heart surgery was detected through postoperative cardiac catheterization in.4 months later. Emergent operation for closure of PDA was performed on the day of recatheterization. After that, patient`s heart failure was easily controlled without any notable problem.[KTCS 1982;2:243-249]