Introduction: Haemophilia is an inherited genetic disorder of coagulation, caused by a lack of specific factor of clotting. Haemophilia is a rare disorder, which is difficult to diagnose and manage. Patients with haemophilia need an additional medical treatment due to the disease itself, but when they also need a surgery, it has to be carefully planned. Purpose: The purpose of the thesis is to present a proper preparation of a haemophiliac for the planned surgical procedure and to define the role of nurses. Methods: These thesis was written by using the descriptive methods with a critical review of Slovenian and foreign literature. The articles used were found with help of online databases and the bibliographic catalogue database COBIB.SI. In the research, 40 different articles of the scientific literature were found, written in the range of last 12 years, and chosen according to the inclusion criteria. Discussion and conclusion: Classic haemophilia or haemophilia A is an inherited disorder of blood coagulation due to the lack or insufficient activity of the factor VIII. A surgical procedure must be, in the case of haemophilia, even more planned as the procedures in the case of people who don’t suffer from the disease mentioned. The team that participates in the surgical procedure has to have experience with patients with bleeding disorder. The nurse plays an important role, because she must be present with the patient all the time. She must carry out her work professionally and according to the ethical rules. In the reviewed literature, no description of the role of nurses in the preparation of a patient with haemophilia in surgery was found.