beta-Thalassaemia is an inherited haemoglobin (Hb) disorder resulting in chronic haemolytic anaemia. The most anaemic patients require regular red blood cell (RBC) transfusions for survival but iron accumulation leads to multisystem dysfunction. Heart complications represent the leading cause of mortality in beta-thalassaemia patients. In this case report we present a successful mitral valve replacement (MVR) in a patient with a severe form of beta-thalassaemia.