Light chain deposition disease (LCDD), belonging to monoclonal gammopathy, refers to monoclonal immunoglobulin light chain deposition in kidney, heart, liver, and other tissues. Most LCDD patients with renal involvement at onset have chronic renal function insufficiency. Typical LCDD-related kidney damage presents relatively specific clinical manifestations. The serum or urine protein electrophoresis and quantitative analysis of free light chains are helpful in diagnosis of LCDD. LCDD-related kidney damage can be confirmed by light microscopy, fluorescence microscopy, and electron microscopy examinations. The treatment of LCDD-related renal impairment should be given in consideration of the degree of B cell proliferation and the renal function impairment caused by monoclonal immunoglobulins. B cell proliferation should be controlled actively with less toxic agents to the kidney such as bortezomib or, if necessary, with a high dose of melphalan plus peripheral blood stem cell transplantation (HDM/PBSCT) program.