Abstract: Thrombophilia is a pathological state characterized by a tendency towards thrombosis and thromboembolism caused by various genetic or acquired factors, either alone or in combination. In kidney transplantation, recipients with thrombophilia have a higher risk of thrombosis and acute rejection, which seriously affects the survival rate of both the recipient and the graft. Risk assessment, early diagnosis and appropriate intervention are crucial for the management of thrombophilia related to kidney transplantation. This article aims to enhance clinicians' understanding and treatment level of thrombophilia related to kidney transplantation by summarizing its epidemiology, common causes and pathogenesis, as well as management strategies.