A Case of Kearns-Sayre Syndrome with Atrioventricular block

A 14-year-old male patient diagnosed as Kearns-Sayre syndrome with chronic progressive external ophtalmoplegia, primary retinal degeneration and type I diabetes mellitus was hospitalized because of cardiac involvement. Having atrio-ventricular complete block permenant pacemaker was prophylactically, since the patient showed a rare mitochondrial myopathy, the case is presented and literatüre is reviewed.