Objective To investigate the clinical characteristics of reversible posterior leukoencephalopathy syndrome (RPLS) with HELLP syndrome and to improve the knowledge of this disease.Methods A retrospective study was performed on 5 cases of RPLS with HELLP syndrome (1 case from our hospital and 4 from literature in China) to analyze clinical manifestations, laboratory and imaging examinations, treatment outcomes, and prognosis. Results Four patients presented with severe hypertension, 5 patients with headache, 5 patients with epileptic seizures, 5 patients with vision disorders, 5 patients with consciousness disorders, 2 patients with mental disorders, 3 patients with focal neurological signs, and 1 patient with limb edema. The laboratory testing found hemolysis, elevated liver enzymes, and reduced platelets among all patients. The 5 patients presented with bilateral white matter abnormalities mainly located in the occipital and parietal lobes, partly invading the frontal and temporal lobes, which appeared as low density on CT images, hypointense on T₁-weighted MRI images, and hyperintense on T₂-weighted MRI images.Conclusions RPLS with HELLP syndrome is a rare complication among patients with pregnancy-induced hypertension. Understanding of the clinical and imaging characteristics will contribute to timely diagnosis and treatment of this disease.