Extracellular matrix (ECM) molecules and their receptors exert a dynamic role in cell-matrix interactions during kidney development and repair processes. They provide a physical substratum for the spatial organization of the cells, but also regulate cell growth and proliferation by interacting with growth factors. In addition, they can regulate signal transduction pathways by binding to integrins or by modulating the activity of signaling molecules such as Wnts. ECM and ECM-related molecules control multiple (if not all) steps of kidney development, including ureteric bud branching morphogenesis, mesenchymal condensation, nephron formation, terminal differentiation of renal tubules, and glomerular basement membrane assembly. Their role still needs to be better documented in renal repair. The emergence of conditionally mutated mice for basement membrane components will provide a useful tool to demonstrate further the involvement of ECM and ECM-related proteins in development and repair.