Coronary artery to coronary sinus fistula (CACSF) in association with stenosis of coronary sinus (CS) ostium and retrograde drainage via persistent left superior vena cava (LSVC) is an extremely rare anomaly presenting in the neonatal period. We report herein a 2-day-old boy with similar anomalies, who presented to us with congestive heart failure and underwent surgical repair. Echocardiography including Doppler ultrasound was a non-invasive, quick and accurate tool to diagnose each abnormality satisfactorily. This anomaly has clinical, physiological and surgical implications due to presence of altered flow pattern and variable anatomy. Therefore, such malformations need a careful assessment, preferably by non-invasive diagnostic tools, and appropriate management.