Haemostasis disorders are serious pathologies that could put dental and surgical procedures at risk as they are associated with postoperative bleeding, which in some circumstances could be prolonged and dangerous for the patient. In-depth knowledge of the problems associated with coagulation pathologies and the suitable specific procedures should be implemented in dental practice. A good awareness of the clinical protocols to be used in these circumstances may help reduce operator stress and increase patient compliance. Collaboration with the haematologist is always recommended to establish an adequate treatment plan, both regarding the administration of therapies that promote haemostasis and for assessing the operative risk. Hereby, we summarize the congenital and hereditary pathologies that lead to haemostasis disorders, which can be found in patients undergoing dental procedures. The purpose of this narrative review is to frame the diseases from a clinical, anamnestic, and etiopathological standpoint, as well as to evaluate an operative approach to the pathology under consideration, with particular attention to anaesthesia manoeuvres and post-surgical haemostasis, to avoid hematoma formation and uncontrolled bleeding which can lead procedure failure up and even death. Of note, it is likewise important to educate the patient about prevention, to keep the oral cavity healthy and avoid invasive procedures, limiting the number of operative sessions.