Congenital pseudoarthrosis of the clavicle is a rare disorder of the shoulder girdle, with only approximately 200 individual cases having been reported in the world literature to date. A persistent left superior vena cava (SVC) is the most common thoracic venous anomaly, and has been observed in 0.3% of the general population. Mesocardia and an aneurysm of the main pulmonary artery, associated with bicuspid pulmonary valves, are both extremely rare entities. We report the first case of a 23 year-old man with the above mentioned complex congenital anomaly.