Disorders of blood coagulation can lead to manifest spontaneous bleeding and an increased risk of bleeding during surgical procedures and interventions. Pathophysiologically, a distinction can be made between defects in primary haemostasis, which lead to impaired platelet adhesion and platelet aggregation, and disorders of secondary (plasmatic) haemostasis, which are characterised by impaired fibrin formation or fibrin stabilisation. Aetiologically, a distinction can be made between rare genetically-determined hereditary defects and common acquired coagulation disorders, which may be based on different pathomechanisms. This overview is intended to provide ophthalmic surgeons with a basis for the perioperative management of patients with genetically determined coagulation disorders undergoing ophthalmic surgery. As there are no specific recommendations in this regard, the recommendations are based on the procedure for other surgical interventions, taking into account the specific bleeding risk associated with ophthalmic surgery.