Kearns Sayre syndrome (KSS) is a rare mitochondrial myopathy that is associated with progressive impaired ventilatory drive, heart block, and peripheral neuropathy. Charcot-Marie-Tooth disease (CMT) is a rare chronic motor and sensory peripheral neuropathy which includes muscle weakness and restrictive pulmonary impairment. Patients with either condition having anesthesia can have postoperative respiratory failure. We report a 52-year-old man diagnosed with variants of KSS and CMT presenting for supraventricular tachycardia (SVT) ablation. This is the first report of successful perioperative management of a general anesthetic in a patient with both KSS and CMT.