The effect of plasmapheresis (PP) monotherapy was evaluated in a 77 year old man with crescentic glomerulonephritis (CrGN). Because both anti-neutrophil cytoplasmic myeloperoxidase autoantibody (MPO-ANCA) and circulating immune complex (IC) were positive, removal of these substances was expected to be beneficial. Thus, the treatment was started with PP alone. During 2 month's time, three series of PP monotherapy were performed: three sessions of plasma exchange (PEX) with fresh frozen plasma (FFP); two sessions of double filtration plasmapheresis (DFPP); followed by another two sessions of PEX. The ANCA remained suppressed for 4 weeks after the first PEX, and increased thereafter. Subsequent DFPP caused a rebound in ANCA titer, whereas the second PEX suppressed ANCA for 1 week. Although creatinine clearance (Ccr) improved from 9.3 to 15 ml/min after the first PEX, and this level was maintained, ANCA increased again after the second PEX. Therefore, the patient was treated with methylprednisolone semi-pulse therapy (500 mg/day for 3 days), followed by mild "cocktail" therapy. In 2 weeks ANCA and IC became negative, and Ccr further improved to 20 ml/min. Thus, PP, when performed alone, was shown to be partially effective, arresting the progression of renal damage in the present case. These observations also argue for the advantage of PP, especially PEX, as an adjunct therapy to immunosuppressive therapy in CrGN.