The persistent left-sided superior vena cava (PLS-SVC) is the most frequent congenital anomaly of the mediastinal veins system. The concomitant agenesis of the right-sided superior vena cava (RS-SVC) is much less frequent. This variant is often discovered incidentally and could be mistaken for other findings or complicated intravascular accesses. A 29-year-old Caucasian woman presented with a decompensated autoimmune cirrhosis and hyperbilirubinemia. After a successful liver transplantation, a central venous catheter (CVC) was placed through the right jugular and a chest X-ray examination demonstrated its position into the left subclavian vein. After some days, it has been replaced by another CVC positioned from the left jugular vein. Using ultrasonography during the positioning maneuvers, we observed the metallic guide of the CVC in what seemed to be the dilated coronary sinus (CS). Subsequent chest X-ray examination demonstrated the catheter's tip in the left paramediastinal position. In order to confirm the catheter's exact position, we chose to inject contrast medium through the CVC under fluoroscopic guidance; the examination confirmed the presence of a PLS-SVC. These findings were also visible in a computed tomography (CT) examination previously performed in another hospital, which demonstrated the agenesis of the RS-SVC as well. We demonstrated a rare case of young patient with a PLS-SVC and agenesis of RS-SVC who underwent major surgery (liver transplantation) without any intra-or post-operative complications, even if advanced venous accesses with high flow catheters were used.