Kidney involvement in immunoglobulin-related amyloidosis (AIg) is common. Although patients with renal-limited AIg tend not to have the high mortality that patients with cardiac amyloidosis have, they do experience significant morbidity and impact on quality of life. The complexity of the pathogenesis remains incompletely understood. Models have been established to prognosticate and assess for the response to therapy. Patients with advanced renal impairment from immunoglobulin light chain amyloidosis still have poor renal prognosis, and better therapy is needed in order to preserve kidney function. Patients who develop end-stage renal disease can undergo renal replacement therapy with kidney transplantation.