Despite its initial description more than 20 years ago, combined orthotopic heart and liver transplantation is still performed infrequently. We report a 53-year-old man with familial hypertrophic restrictive cardiomyopathy who presented with right heart failure and end-stage liver failure and required combined orthotopic heart and liver transplantation. Rather then using a staged approach, the surgery was performed using a technique of simultaneous implantation supported by cardiopulmonary bypass. The relative merits of using a simultaneous approach are discussed. The patient had an uneventful postoperative course and went home on postoperative day 14.