Membranoproliferative glomerulonephritis (type 1) associated with infected ventriculoatrial shunt was observed in an 11-year-old girl. The removal of the shunt and antimicrobial therapy led to rapid improvement in the clinical symptoms, serologic abnormalities, and glomerular alterations. Serum complement levels were initially low and returned to normal values within 1 month. Urinary sediment abnormalities resolved within a few months. Cultures taken from cerebrospinal fluid and the end of the shunt grew Staphylococcus epidermidis. Initial renal biopsy obtained immediately before shunt removal showed membranoproliferative glomerulonephritis (type 1). Repeat biopsy performed 2 years after shunt removal showed moderate improvement of glomerular alterations. This study of pathologic and laboratory changes following therapy suggests that the pathophysiology of the nephritis was based on an immunologic process, and that removal of antigen resulted in reversal of glomerular changes.