We report a normotensive case of reversible posterior leukoencephalopathy syndrome caused by transient hypercoagulable state. Hypertension is the main risk factor for reversible posterior leukoencephalopathy syndrome, which is believed to occur as a result of high blood pressure-related dysfunction of cerebrovascular endothelial cells, because it commonly appears in hypertensive emergency. However, in this completely normotensive case, the typical clinical findings of reversible posterior leukoencephalopathy syndrome were triggered by transient hypercoagulable state without any blood pressure variation. The case was successfully treated with anticoagulation therapy using heparin. Thus, this case indicates that reversible posterior leukoencephalopathy syndrome is induced by cerebrovascular endothelial dysfunction, which is induced not only by high blood pressure but also hemostatic dysfunction.