Objectives　To explore the pathogenesis, diagnosis, treatment and prognosis of children with Henoch-Schönlein purpura nephritis (HSPN) complicated by posterior reversible leukoencephalopathy syndrome (PRLS). Method　The clinical data of 2 children with HSPN complicated by PRLS were retrospectively analyzed, and the related literature was reviewed. Results　Two boys aged 8 years and 14 years were given hormone, immunosuppressant, gamma globulin, plasma and albumin because of obvious edema and proteinuria. Seizures and loss of consciousness occurred during the course of the disease. MRI showed multiple abnormal signals in frontal lobe, parietal lobe and occipital lobe. The seizure stopped and consciousness recovered after treatment of controlling convulsion and decreasing intracranial pressure, and MRI abnormal signal was also significantly improved. Conclusion　Clinicians should be vigilant about PRLS during the treatment of HSPN.