Total knee replacement in patients with haemophilia A is a challenging procedure with high risk of complications. Due to the massive destruction of the joint and significantly reduced range of motion, total knee replacement should be performed only by a surgeon with high degree of expertise and experience. During the perioperative period, patients require factor VIII (FVIII) replacement therapy supervised by a haematologist, under control of plasma activity levels. Possible early complications include delayed wound healing, soft tissue and joint bleeding, development of pseudoaneurysm and early infection. Once complications occur, prompt detection and introduction of proper treatment is fundamental.