Perioperative dilutional coagulopathy is a major coagulation disorder during adult and pediatric surgery. Although the main underlying mechanisms are comparable, data of the development and management of dilutional coagulopathy in children are scarce. Observational data showed that intraoperative coagulation disorders mainly based on complex disturbances of clot firmness including acquired fibrinogen as well as factor XIII deficiencies, while clotting time and platelet counts remained fairly stable. A fast and reliable monitoring of the entire coagulation process (e.g. thrombelastometry) might be of extreme value for detection and guidance of effective coagulation management. Although the transfusion of fresh frozen plasma was recommended in several guidelines, the use of coagulation factors might offer an alternative and potentially superior approach in managing perioperative coagulation disorders. Further studies are urgently needed to determine the efficacy of modern coagulation management.