Herein, we report a case of fibrillary glomerulonephritis (FGN). FGN usually shows non-amyloidal fibrils in the mesangium and glomerular capillary walls on electron microscopy. Inherited cases of FGN have been reported in only 3 families, and the suspected genetic form was autosomal dominant. In the present case, the deposition of microfibrils in the arteriolar wall as well as the glomerulus is unique. Our patient's father died of nephrotic syndrome, and his elder brother had a biopsy-proven glomerulopathy. The histological findings of the brothers are similar to mesangial proliferative glomerulonephritis and resemble each other. Therefore, our case is presumed to be familial FGN. Additionally, herein, we review the literature and reconsider the histological and clinical characters of FGN.