AL amyloidosis is a systemic disease caused by the deposition of fibrillar proteins in organs and tissues. These deposits are responsible for organic dysfunctions and may be rapidly lethal. Current therapy is unsatisfactory. We describe three clinical cases of multisystemic AL amyloidosis but with different initial presentation: nephrotic syndrome, cardiac failure and hepatic insufficiency. We review the literature about the clinical features, diagnostic methods and treatment of the disease.