Background: Posterior reversible LEUKOENCEPHALOPATHY syndrome (PRLS) is a clinical and radiological syndrome of heterogeneous etiologies that are grouped because of the similar findings on neuroimaging studies which may occur at any age. The pathogenesis of RPLS remains unclear, but it appears to be related to disordered cerebral autoregulation and endothelial dysfunction. The syndrome is more commonly seen in women, RPLS has been described in several medical conditions, with hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressant drugs being the most common conditions. Prompt recognition and treatment are important in preventing the permanent damage that can occur in this otherwise typically reversible condition. The pathogenesis of RPLS remains unclear, but it appears to be related to disordered cerebral autoregulation and endothelial dysfunction. This report aimed to introduce a case of reversible posterior LEUKOENCEPHALOPATHY following postpartum thrombotic thrombocytopenic purpura (TTP). Case presentation: The patient was a 30-year-old primigravid woman at 33 weeks of pregnancy who was referred to the Ghaem hospital, Mashhad University of Medical Sciences in Mashhad in July 2017 due to high blood pressure, blurred vision, headache and generalized tonic colonic seizure. She did not report in previous history of high blood pressure or seizure before pregnancy. With a diagnosis of eclampsia remote from delivery, she underwent a cesarean section. After delivery, generalized tonic colonic seizure repeated several times and a significant reduction in consciousness level happened. Renal failure also occurred, so she underwent daily plasmapheresis with a diagnosis of TTP. After 35 days, she was discharged with a good general condition. Conclusion: In predisposing conditions, such as high blood pressure in pregnancy, in the case of clinical suspicion of posterior LEUKOENCEPHALOPATHY, any attempt for early diagnosis and appropriate treatment are important factors in reducing the rate of morbidity and mortality.