Objective　To explore the clinical characteristics of renal disease with posterior reversible encephalopathy syndrome (PRES) in children. Methods　The clinical data of 6 children confirmedly diagnosed of kidney disease with PRES from 2015 to 2018 were retrospectively analyzed, and the relevant literature was reviewed. Results　In 6 children (4 boys; 2 girls) aged from 3 to 14 years, there were 2 cases of nephrotic syndrome. Two cases of purpura nephritis, one case of Henoch-Sch?nlein purpura and one case of lupus nephritis, All had a history of hormone and immunosuppressant therapy. Clinical manifestations included acute onset in all, convulsions, loss of consciousness, elevated blood pressure, abnormal mental behavior, vomiting, headache, and visual disorders. The imaging findings were characterized by reversible white matter abnormalities, mostly located in the posterior hemisphere. Five children recovered to their pre-morbid state and one died. Conclusions　PRES is common in children with renal disease treated with hormones and immunosuppressants, and the prognosis is generally good, but not completely reversible.