A 28-year-old woman was admitted due to fever, dyspnea, hematuria and acute renal insufficiency. 1 months ago, she had been diagnosed as having ventricular septal defect with mild dyspnea and chronic cough. Infective endocarditis (IE) was diagnosed by clinical findings including vegetation in echocardiography. A blood culture drawn during the hospitalization grew streptococcus sanguis. A renal biopsy showed IE-induced crescentic glomerulonephritis (GN). Antibiotic treatment alone was effective for fever, but not for gross hematuria and renal insufficiency. After the initiation of plasmapheresis, gross hematuria and acute renal insufficiency was dramatically improved. After clinical stability was achieved, closure of the ventricular septal defect was performed. This result suggests that plasmapheresis may be beneficial in the treatment infective endocarditis-induced crescent GN.