Amyloidoses form a group of diseases in which extracellular insoluble fibrils cause organ injury and failure. These aggregates are built of misfolded proteins. Current amyloidosis classification is based on the type of the precursor amyloid protein. Distribution of the amyloid fibrils can be localized or systemic. The two most common systemic diseases are: amyloid light chain (AL) amyloidosis, being a consequence of clonal expansion of plasma cells in the bone marrow, and amyloid A (AA) amyloidosis, secondary to chronic inflammatory disorders. Renal involvement remains a frequent clinical manifestation of both of them with clinical pattern of massive proteinuria and progressive renal failure. Early diagnosis and treatment is essential because of the progressive character of the disease. The goal of current treatment approaches is to decrease the amount of amyloidogenic proteins and depends on its type. Systemic amyloidosis remains a disease with poor prognosis, especially in patients with cardiac involvement.