A 44-year-old female admitted to our hospital because of fever, purpura and macroscopic hematuria. She had been diagnosed as having ventricular septal defect (VSD). She noticed purpura with pain on bilateral legs and macroscopic hematuria since September 18, 1994. Three weeks later she also manifested a fever. Physical examination of admission revealed numerous purpura and leg edema. Laboratory data showed macroscopic hematuria, marked anemia (Hb 3.3 g/dl), leukocytosis, azotemia (Cr 2.7 mg/dl) and positive acute phase reactants. Increased serum immune complex level and hypocomplementemia were also found. The diagnosis of allergic purpura was made initially, but positive blood culture of Streptococcus mitis and the detection of vegetation attached to the right ventricular wall near the ostium of the VSD made the difinite diagnosis of infective endocarditis (IE). Chemotherapy with PCG was started for two weeks but with no effect. The chemotherapy was altered to panipenem/betamipron with a daily dose of 3 g, Then, her fever fell and purpura, macroscopic hematuria and renal failure gradually disappered. In this case, the cause of renal manifestations was considered to be immune complex glomerulonephritis. This is the first report of IE with macroscopic hematuria due to immune complex glomerulonephritis.