Stem cell-based therapies have raised considerable interest to develop regenerative treatment for neurological disorders with high disability. In this review, we focus on recent preclinical and clinical evidence of stem cell therapy in the treatment of degenerative neurological diseases and discuss different cell types, delivery routes and biodistribution of stem cell therapy. In addition, recent advances of mechanistic insights of stem cell therapy, including functional replacement by exogenous cells, immunomodulation and paracrine effects of stem cell therapies are also demonstrated. Finally, we also highlight the adjunction approaches that has been implemented to augment their reparative function, survival and migration to target specific tissue, including stem cell preconditioning, genetical engineering, co-transplantation and combined therapy.