Abstract: The integrity of human corneal epithelium is maintained by the continuous renewal of limbal epithelial stem cells. Limbal niche-the special region of corneal limbus,  is the microenvironment that stem cells live on. The limbal niche is composed of a variety of cells,  signal molecules and extracellular matrix. The coordination of all the components in the niche maintains the homeostasis of limbal niche and promotes the proliferation,  migration and differentiation of limbal stem cells. Any disturbance to the homeostasis of limbal niche,  including congenital,  traumatic,  and immune diseases,  would lead to dysfunction of stem cells. For patients with limbal stem cell deficiency,  the reconstruction of limbal niche through the regeneration of extracellular matrix and the application of bioactive factors and mesenchymal stem cells,  is of great significance for the recovery and long-term maintenance of stem cells. (Int Rev Ophthalmol,  2020,  44:  391-396)

Key words: limbal niche, limbal epithelial stem cells, extracellular matrix, signal pathway, reconstruction of the limbal niche