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Total drainage of systemic blood into the left atrium is an exceptional finding in absence of severe intracardiac malformation. In our patient, a boy 3 years and 4 months old, the left superior vena cava, the inferior vena cava, and the coronary sinus drained into the left atrium; there was no right superior vena cava, and the only associated anomaly was a large atrial septal defect. This rare form of cyanotic congenital heart disease was correctly diagnosed and successfully corrected.
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