Coagulation disorder, also known as coagulopathy, is commonly associated with traumatic brain injury. The exact mechanisms underlying their association are poorly understood although multiple factors, including abnormalities of coagulation system, hypoperfusion, dilution, metabolic acidosis, hypothermia and inflammation, have been proposed. The abnormalities of coagulation system mainly include tissue factor release, hyperfibrinolysis, and platelet abnormalities. Recently, the dominant role of tissue factor has been challenged by hypoperfusion and the related protein C signaling pathway. The role of inflammation is gradually acknowledged in the late stage of traumatic brain injury-associated coagulopathy. This review was aimed to discuss the above pathogenesis.