Rare bleeding disorders (RBDs) account for 3-5% of inherited bleeding disorders and are caused by deficits in various hereditary coagulation factors such as factor I, II, V, VII, X, XI, XIII, combined factor V and VIII, and vitamin K-dependent protein deficits. Common symptoms of the RBDs include dental caries, gingivitis, postoperative bleeding, hematomas, and hemarthrosis. The latter two symptoms are evident in deficiencies of prothrombin (factor I) and factor X. Therapy and control of affected patients cannot be based on a rigid protocol; each hematological disease and each individual requires a unique strategy that requires the dentist to take into account several factors such as the type of inherited hematological disorder, the treatment strategy for the disorder, an assessment of whether routine treatment should be modified to facilitate dental treatment requirements, and an assessment of whether such an alteration will require replacement therapy or drug treatments capable of raising the defective factor level. In addition, the location and extent of dental surgery and the treating dentist’s expertise are to be taken into consideration. Depending on the inherited coagulopathy, there are four treatment options: coagulation factor replacement therapy, desmopressin, antifibrinolytic medications such as tranexamic acid, and local hemostatic treatments.