The term "amyloidosis" covers a group of conditions characterised by extracellular protein deposits in the form of insoluble fibrils with a beta-pleated sheet structure. Systemic amyloidoses very often involve the kidney, as the primary AL (Ig light chains), the secondary AA (amyloid A), but also some hereditary forms of amyloidosis. The main renal symptom of amyloidosis is proteinuria. In addition to histological work-up using lightmicroscopy, immunohistochemistry and electronmicroscopy, diagnosis often calls for amyloid classification by means of DNA sequencing or laser microdissection with mass spectrometry. The therapeutic approach depends on the underlying primary disease but especially in AL amyloidosis new chemotherapeutic options and interesting data from bone marrow as well as solid organ transplantation have recently become available.