End-stage heart failure (ESHF) represents the leading cause of death for adult congenital heart disease (ACHD) and heart transplant (HT) remains the first-line treatment. Although many patients are ineligible for transplantation due to advanced comorbidities, left ventricle assist devices (LVAD) is considered a valid option. Herein we present a case of dextrocardia in situs viscerum inversus totalis (SVIT) who was implanted with LVAD HeartMate II ten years ago. To the best of our knowledge, this was the first case of dextrocardia treated with a totally intrathoracic device and he is to date the longest survivor with a HeartMate II in SVIT.