In routine diagnosis on renal biopsy, one of the confusing fields for pathological diagnoses is the glomerulopathies with fibrillary structure. The primary glomerulopathies with a deposit of ultrastructural fibrillary structure, which are negative for Congo-red stain but positive for immunoglobulins, include fibrillary glomerulonephritis and immunotactoid glomerulopathy. Several paraproteinemias including cryoglobulinemia, monoclonal gammopathy, and light chain deposition disease as well as hematopoietic disorders including plasmacytoma, plasma cell dyscrasia, and B cell lymphoproliferative disorders involve glomerulopathy with an ultrastructural fibrillary structure. A rare glomerulopathy with fibrillary structure that stains negative for Congo-red as well as for immunoglobulins has been also reported. The pathological diagnoses of these glomerulopathies can include either glomerular diseases, or paraproteinemic diseases, or hematopoietic diseases. The terminology is still confusing when glomerular diseases can be combined with paraproteinemic diseases and/or hematopoietic diseases. Therefore, the generic term, ‘glomerular deposition disease’ (GDD), has been proposed by pathologists with a requirement for clinicians to detect autoantibodies, paraproteins as well as to carry out a bone marrow check. An attempt has been made to rearrange the diseases with related disorders of fibrillary deposits, based on detailed clinical and pathological finding and to elucidate the correlation between GDD, paraproteinemia, and hematopoietic disorder.