This document summarizes a presentation on kidney progression in Fabry disease. It discusses the natural history of Fabry kidney disease and pathology. Studies show a progressive decline in kidney function with age for untreated males and females with Fabry disease. Kidney pathology involves the accumulation of globotriaosylceramide inclusions in various kidney cell types including podocytes. Enzyme replacement therapy can help clear inclusions, but not completely, and higher doses of ERT may lead to better renal outcomes than lower doses. Early treatment may provide long-term renal benefits.