Limbal stem cell deficiency (LSCD) is a rare but often progressive condition associated with significant ocular morbidity and visual loss. LSCD can be partial or total and can affect one or both eyes, important factors to consider when planning disease management. Decades of laboratory and clinical research have provided the basis for contemporary understanding and management of this condition. In this narrative review we highlight the important breakthroughs in limbal stem cell science which have shaped limbal stem cell therapy, the current cell culture techniques, and the respective merits of the substrates used. We will outline the key management principles in the treatment of LSCD where a multi-disciplinary approach is essential in optimising outcomes and describe the current clinical and surgical options available. We will also explore the emerging treatments on the horizon.