Haemophilic pseudotumour (HP) is a rare but very serious complication of haemophilia. HP affects mainly patients with severe haemophilia and those who have developed antibodies to factor VIII or factor IX. We report on a 45-year-old man with haemophilia A and high titres of inhibitors who developed an extensive HP with progressive destruction of the right ilium over a period of 12 years. The different therapeutic options (conservative management by replacement therapy, surgical approach, radiotherapy, percutaneous evacuation with secondary refilled cavity and transcatheter arterial embolization) are reviewed.