Advances in the diagnosis and management of congenital heart disease (CHD) have resulted in an increased number of patients living into adulthood. Despite increased survival, these patients often require surgery at a young age and are susceptible to developing complications related to the degeneration of surgical prostheses and valves. This case describes to our knowledge, the first successful balloon expandable valve-in-valve transcatheter aortic valve implantation (ViV-TAVI) for stenosis of a BioValsalva stentless aortic graft in a patient with complex congenital heart disease and two prior sternotomies. Prior to ViV-TAVI, the patient was critically ill and unable to be weaned from intensive care unit supports due to recurrent pulmonary oedema. Our case demonstrates successful ViV-TAVI in what was considered an extreme-risk patient. This procedure enabled the patient to be rapidly weaned from respiratory supports and discharged seven days post-procedure.