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We describe the case of a 1-month-old infant with a complete atrioventricular septal defect with right dominance, situs solitus, and drainage from the persistent left superior vena cava to the coronary sinus. Corrective surgery was carried out without previous cardiac catheterization. During the operation, the right superior vena cava was found to be absent. Cyanosis and head-and-neck edema were observed in the immediate postoperative period. Transthoracic echocardiography carried out after injection of a small volume of stirred saline into an epicranial vein demonstrated the presence of microbubbles in the left cardiac cavities. A second operation was performed to prevent drainage from the left superior vena cava to the left atrium (via the unroofed coronary sinus) and to insert a PTFE conduit between the innominate vein and the right atrial appendage. The outcome was excellent. In this report, the embryological, clinical, diagnostic and therapeutic characteristics of this entity are discussed.