Amyloidoses are rare diseases, defined by the accumulation of extracellular deposits with ultrastructural fibrillary organization, and molecular beta-pleated conformation. Amyloidoses are defined by the type of protein aggregates, the most common being immunoglobulin light-chains amyloidosis (AL). The treatment of AL amyloidosis has recently been improved by serum immunoglobulin light chains assay for close treatment monitoring; prognostic markers of cardiac damage such as BNP, NT-proBNP and troponin; and the emergence of new anti plasma cell drugs. AA amyloidosis should be screened by a search of proteinuria in every patient with chronic inflammatory disease. To diagnose rare hereditary amyloidoses requires to gather clinical, pathological and genetic data. Recent therapeutic advances showed efficacy of a molecule stabilizing transthyretin in early forms of amyloid neuropathy due to transthyretin mutations.