We have studied a girl seven and a half years old with a renal disease manifested by proteinuria and nephrotic syndrome combined with initial macroscopic hematuria normal blood pressure and persistent depression of the complement C3 levels with the complement activating gamma globulin (C3 Nef) present in serum. The anatomopathological study showed findings of membranoproliferative glomerulonephritis type II with intramembranous deposit (dense deposit disease). An unusual association with partial lipodystrophy was present in this case. A follow-up of five years, and a few considerations about etiopathogenic theories were presented.