Objective To investigate the clinical and imaging features and etiology of reversible posterior leukoencephalopathy syndrome (RPLS).Methods A retrospective analysis was performed on the clinical and imaging data and the treatment process of 38 patients with RPLS who were admitted to the Xiangya Hospital, Central South University, from 2009 to 2014.Results The 38 RPLS patients included 23 females and 15 males. There were 36 cases having a history of hypertension, and 6 having a history of eclampsia or pre-eclampsia. Additionally, 5 had immunosuppressant and 3 had a history of chemotherapy. Headache and dizzy were the primary symptoms among 30 patients (78.9%), and blood pressure increase, nausea and vomiting, blurred vision, epileptic seizure, and disturbance of consciousness were the most common clinical symptoms. Cranial magnetic resonance imaging revealed symmetric long T₁ and T₂ signals in bilateral white matter of the posterior cerebral hemisphere, with high FLAIR and low DWI signals; cerebral cortex was commonly involved.Conclusions RPLS is an encephalopathy syndrome with good prognosis and complex etiology. The diagnosis of RPLS mainly relies on clinical and imaging features.