We present a case of a 68-year-old man with anti-glomerular basement membrane (anti-GBM) antibody-induced glomerulonephritis accompanied by periglomerular granulomatous reaction and massive eosinophilic infiltration. Periglomerular granulomatous giant cells were derived from macrophages, shown by positive staining for monoclonal antibody against cluster of differentiation 68. Staining for eosinophil cationic protein indicated that activated eosinophils were involved in the tubulitis, as well as in the glomerular injury. The patient was admitted to the hospital with fever, loss of appetite, edema of the extremities, abnormal urinalysis results, and rapid progressive renal failure. At an examination 8 months before admission, his serum creatinine level (1.0 mg/dL [88.4 micromol/L]) and urinalysis results were normal. On admission, an elevated serum creatinine level (5.1 mg/dL [450.8 micromol/L]) and marked eosinophilia (eosinophils, 5.00 x 10(3)/microL [5.00 x 10(9)/L]; 37.2% of total white blood cell count) were observed. Serum anti-GBM antibody titer was high (43 EU), measured by means of an enzyme-linked immunosorbent assay. No respiratory or ophthalmological abnormalities were seen. Intravenous steroid pulse therapy followed by oral prednisolone (PSL) was effective for reducing the fever, eosinophilia, anti-GBM antibody titer, and C-reactive protein level, but did not improve renal function because renal tissue already was irreversibly damaged. Oral PSL dose was tapered off without relapse. The patient underwent long-term hemodialysis therapy, which dissipated the edema. He was discharged from our hospital 65 days after admission. Three months later, his anti-GBM antibody level was less than 10 EU, and the number of peripheral eosinophils stayed with the normal range.