We present a 22-year-old male patient, a history of a right bidirectional Glenn procedure (BDG), with dyspnea, cyanosis, palpitations and chest pain. Transthoracic echocardiography and computed tomography showed aneurysmal dilation of the right superior venous system. BDG takedown was performed with resection of the aneurysmal superior vena cava and reconnection of the superior vena cava to the right atrium and the creation of a central systemic-to-pulmonary shunt. The patient died from complications derived from a massive collateral circulation.