A 73-year-old female was admitted to our hospital for high fever, malaise, anorexia, anuria, and systemic edema. Following several examinations, we started antimicrobial therapy and hemodialysis for acute kidney injury due to suspected bacteremia on the first day. However, her condition did not improve, and laboratory data revealed normocytic-normochromic anemia and thrombocytopenia on the sixth day. On the eighth day, these abnormalities progressively worsened. Increased serum LDH, decreased serum haptoglobin, negative results for both direct and indirect Coombs tests, and schistocytes in the peripheral smear suggested intravascular hemolysis due to thrombotic microangiopathy. We started plasma exchange (PE) using FFP. On the twelfth day, anti-glomerular basement membrane (GBM) antibody in her serum at admission was found to be positive. We diagnosed her with acute kidney injury associated with anti-GBM antibody disease and TMA. We added steroid pulse therapy and continued PE and hemodialysis. Although we could induce TMA remission following twenty-five sessions of PE, her renal function did not improve. To our knowledge, there are only eleven reported cases of anti-GBM antibody disease associated with TMA. Only four of these eleven cases were successfully treated, and five cases died. We consider that this case is quite rare.