A 12-year-old who had a ventriculoatrial shunt for hydrocephalus due to congenital aqueduct stenosis and later developed shunt nephritis is reported. His renal function detoriated progressively within eight months while he was lost to follow-up. After removing the shunt, and with appropriate aggressive treatment renal function improved and complement levels returned to normal. Early removal of the shunt is mandatory is such a condition but even in delayed cases removal is beneficial.