Kearns-Sayre Syndrome is a multisystemic mitochondrial cytopathy characterized by specific ophthalmic signs, cardiac conduction dis turbances with endocrine, musculoskeletal and central nervous system involvement. As a highly heterogeneous condition, establish ing an accurate diagnosis of this disorder can often be seriously delayed. It usually occurs before the age of 20 with ocular symptoms at first. Cardiac manifestations include progressive degeneration of the conduction tissue, leading to different types of conduction dis turbances, which in many cases are responsible for significant decrease of life expectancy. There is currently no causative therapy avail able for Kearns-Sayre Syndrome patients. Several interventions including ophthalmic or neurological may be necessary in order to im prove the quality of life, however improving prognosis in this group of patients impose prompt recognitions of those, who require early pacemaker implantation. Therefore, the aim of this article is to review the current knowledge about Kearns-Sayre Syndrome in light of the most typical ophthalmic findings, which can handily be detected by cardiologists and applied to accelerate accurate diagnosis and elaborate the most appropriate therapeutic strategies