We describe the case of a 67 year-old female with nephrotic syndrome and rapidly progressive renal failure. The nephropathy was characterized by deposits of randomly oriented fibrils with a diameter of about 18-20 nm on electron microscopy. Immunofluorescence microscopy was performed and there was no staining for immunoglobulins and complement. We diagnosed atypical fibrillary glomerulopathy with absence of immune deposits. The patient developed end-stage renal failure rapidly. We review in the literature new clinical and pathogenetic features related to fibrillary and immunotactoid glomerulopathy.