Today the populations of haemophilia patients in many countries have a higher life expectancy than previously known, and age-related disorders such as arterial disease are expected to become more prevalent, calling for surgical intervention. Cardiac surgery constitutes a major haemostatic challenge because of sternotomy, the need of total heparinization, extracorporal circulation, mild hypothermia and cardiac arrest. To evaluate our current experience and results with cardiac surgery in patients with haemophilia the present case series report on six patients with haemophilia A (Severe = 1, Moderate = 1, Mild = 4) undergoing cardiac surgery (coronary artery bypass grafting; CABG = 2, aortic valve replacement = 1, CABG + aortic valve replacement = 2, ventricular resection + mitral valve reconstruction = 1). The present paper provides detailed information on the haemostatic treatment regimens adopted (factor concentrate dosages, timing and duration) and postoperative thromboprophylaxis (dosing and duration of low molecular weight heparin). Moreover, we present data on concomitant disorders (hypertension, hypercholesterolaemia, atrial fibrillation and diabetes), left ventricle ejection fraction (30-60%), type of anaesthesia, total amount of heparin (34 500-53 500 IU) and duration of extracorporeal circulation (80-115 min). Clinical outcomes included: re-operation because of bleeding (none), transfusion requirements, peri- and postoperative blood loss and complications and postoperative development of inhibitors (none). Clinical outcomes were compared with a control group of patients (n = 5993) without haemophilia and we found no difference in postoperative morbidity. Adopting meticulously supervised haemostatic treatment regimens, we have successfully performed major cardiac surgery in patients with haemophilia A. The clinical outcome as well as the severity and incidence of postoperative complications were similar to patients without haemophilia.