We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (GBM) disease accompanied by positive anti-nuclear cytoplasmic antibody (ANCA) presenting central nervous system involvement in a 43-year-old female. She was treated with combination therapy consisting of corticosteroid pulse therapy, oral prednisolone, cyclophosphamide, plasma exchange, and hemodialysis for end-stage kidney disease. During the treatment, she suddenly developed seizures and consciousness disturbance. She was diagnosed with posterior reversible encephalopathy syndrome (PRES) by magnetic resonance imaging and an IgG oligoclonal band was found in the cerebrospinal fluid, which suggested that there were potentially abnormal antibodies outside the central nervous system, leading to vasculitis or endothelial injury. The PRES was quickly controlled by anti-hypertensive agents and reinforcement of immunosuppressive treatment. According to the literature review, patients with double-seropositivity for ANCA and anti-GBM antibodies are predisposed to PRES; therefore, nephrologists should take precautions due to the potential risk of PRES in these patients.