Treatment of limbal stem cell deficiency is challenging. Multiple options can be adopted according to the underlying cause and the patient and physician preferences. Stem cell transplant is a common treatment modality and several techniques have been described with outcomes varying by the laterality of the condition. Keratoprosthesis is a preferred option for bilateral conditions. Indications for type 1 and type 2 keratoprosthesis differ and the past 2 decades have seen a revolution in the field of keratoprosthesis with encouraging and improved outcomes. Management also includes preventive measures and measures to optimize/stabilize the ocular surface that would go a long way in reducing the deterioration of the ocular surface. The aim of this review is to provide an overview of the currently available techniques and to present a comprehensive algorithm to assist in decision making for unilateral and bilateral limbal stem cell deficiency.