Systemic amyloidosis is a group of disorders caused by the production of amyloid proteins that generate a deposit of these in various tissues, producing a large number of clinical syndromes diseases. To start several pathophysiological mechanisms responsible are known in the formation and deposition of these abnormal proteins, signs and symptoms are very diverse and depend on the organ involved: primary amyloidosis and secondary amyloidosis, which would include all those not caused by abnormal secretion of light chains by plasma cells and be called by type of amyloid precursor: As for classification describes two basic forms. For diagnosis requires biopsies, performing special stains, determination of atrial natriuretic peptide and studies of complementary images, once made should be classified correctly and according to the clinical stage the most appropriate treatment which may include being raised, chemotherapy, to hematopoietic transplantation. As for his prognosis is an entity with high mortality and median survival is two years, patients with coexisting multiple myeloma are the worst prognosis. Cardiac involvement is the main determinant of mortality.