The Ross operation is the procedure of choice for aortic valve disease in paediatric patients, because of the potential for growth of the pulmonary autograft and because anticoagulation is not required. However, early and late autograft dilatation and severe aortic regurgitation may occur. Transcatheter aortic valve implantation (TAVI) is an effective procedure for treatment of severe degenerative aortic stenosis in patients deemed inoperable or high surgical risk. Off-label treatment of severe non-calcified aortic regurgitation with transcatheter heart valves has occasionally been reported. We describe the first case of TAVI for severe aortic regurgitation in a young woman 10 years after a Ross operation. The procedure was performed on a compassionate basis after the patient was deemed inoperable because of severe reactive pulmonary hypertension (95/55/68 mmHg; pulmonary resistance 18.3 UR) and haemodynamic compromise. A 29 mm CoreValve (Medtronic, Minneapolis, MN, USA) was implanted. A second prosthesis was deployed "valve-in-valve" for residual severe paravalvular leak, caused by the peculiar post-surgical anatomy of the left ventricular outflow tract. The procedure was successful and the in-hospital course was uncomplicated. During follow-up, pulmonary pressure and resistances were significantly lowered and at four years the patient showed markedly improved exercise tolerance.