We report the first case of myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA)-associated glomerulonephritis in a patient with CREST syndrome. A 74-year-old Japanese man with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) developed rapidly progressive renal failure without elevation of blood pressure. Renal biopsy revealed glomerular sclerosis and fibrous crescents. The MPO-ANCA titer was elevated to 145 EU/ml. When patients with collagen diseases develop rapidly progressive glomerulonephritis, the possibility of MPO-ANCA-associated glomerulonephritis should be kept in mind.