Light Chain Deposition Disease (LCDD) is a rare monoclonal gammopathy characterized by deposition of light chains along the basement membranes of various organs, leading to progressive dysfunction (1,2). It has histological features that differ from amyloidosis and is most often characterized by the overproduction of kappa light chains (2). The kidney is usually the most affected organ and is responsible for the initial clinical manifestations, whereas liver dysfunction is less common and generally less severe than kidney involvement (1,3). We present a case of LCDD in a patient with renal dysfunction associated with secondary sclerosing cholangitis (SSC) and severe liver and kidney involvement, efficiently treated with autologous stem cell transplantation followed by double liver-kidney transplantation.