Light-chain deposition disease (LCDD) is an uncommon condition in which monoclonal light chains are deposited in different organs causing varying degrees of tissue damage. We report the case of a 56-year-old male with progressive renal failure as the first manifestation of LCDD without initial evidence of monoclonal immunoglobulin protein in either serum or urine. The patient later developed severe systemic disease with rapid multiple organ involvement. Finally, he died 2 months after diagnosis, despite steroid and cyclophosphamide therapy.