Due to the global prevalence of oral disease, tooth extraction is the most common surgical procedure required in general population thus likely to be similarly common in patients with haemophilia, especially those in older age and those living in countries with restricted resources. There are little or no consensus about optimal level and duration of factor replacement (FRP) therapy required to prevent bleeding complication following surgery and low levels of evidence to inform protocols and guidelines. The goal of this article was to review the literature regarding haematological treatment protocols and to assess their effectiveness in prevention of bleeding complications during and after tooth extractions in people with haemophilia. A total number of 29 articles were identified. Only two of the studies were randomized controlled trials, and meta-analysis was not possible. Significant heterogeneity regarding haematological regimes, dental surgical procedures, disease severity and sample size of published studies which are unable to reliably inform the provision of safe dental surgery was noted. Based on the haematological regimens, all studies were classified into one of three groups: pre- and postoperative FRP or DDAVP, single preoperative FRP or DDAVP, and no FRP treatment. The overall reported bleeding rate in case of both pre- and postoperative FRP and single dose FRP preoperative is similar, 11.9% and 11.4%, respectively, indicating that minimizing the use of clotting factor concentrate is possible if proper local haemostatic measures are provided. Strictly designed prospective studies with higher number of patients are necessary to get firm conclusions about optimal FRP treatment required to prevent bleeding complications during and after oral surgery in patients with haemophilia.