A 71-year-old woman was hospitalized for the treatment of fatigue, fever, and cough. On admission, she showed increased serum inflammation markers, severe anemia, pulmonary hemorrhage, and advanced acute kidney injury requiring hemodialysis. Her serum anti-glomerular basement membrane (GBM) antibody titer was found to be extremely high on the 7th hospital day. She was eventually diagnosed with anti-GBM disease. She was treated with a combination of corticosteroid pulse therapy, oral prednisolone and cyclophosphamide, and plasma exchange, but continued to require maintenance hemodialysis for end-stage kidney disease. During her treatment, she suddenly developed headache, blindness, seizure, and consciousness disturbance. She was diagnosed by magnetic resonance imaging with posterior reversible encephalopathy syndrome (PRES) with subcortical cerebral hemorrhage. Both the PRES and cerebral hemorrhage subsided soon after control of her hypertension and reinforcement of immunosuppressive treatment. PRES, particularly when accompanied by cerebral hemorrhage, may cause irreversible and lethal neurological abnormalities, and nephrologists should, therefore, be aware of the potential risk of PRES in patients with anti-GBM disease. We discuss the current case in the light of the previous literature.