Regeneration and repair of the corneal epitheliumCorneal epithelium rely on a population of stem cells residing in the basal epithelial layer at the corneoscleral limbus within a specialized niche microenvironment. Damage to or dysfunction of this population lead to limbal stem cell deficiencyLimbal stem cell deficiency (LSCD), a rare but debilitating and potentially blinding ocular surfaceOcular surface disease, that can be caused by many hereditary and acquired conditions, most frequently ocular burns. Management of LSCD requires an accurate diagnosisDiagnosis including stagingStaging of LSCD severity using clinical, laboratory and in vivo-imaging techniquesTechnique. A variety of medical and surgical procedures, which transfer autologous or allogeneic stem cells onto the diseased eyesEye of LSCD patients, have been developed and clinically validated to reconstitute the corneal epitheliumCorneal epithelium. Given some limitations of established procedures, novel therapeutic approaches are needed. In this chapter, we will discuss the etiology, demographics, clinical manifestation, classification, diagnosisDiagnosis as well as current and future treatmentTreatment modalities of LSCD.