Tehran University of Medical Sciences Archives of Anesthesiology and Critical Care 2423-5849 0 0 2026 02 19 1553 1553 Shilpa Deshmukh Department of Anesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pune, Maharashtra, India. Ashish Erry Department of Anesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pune, Maharashtra, India. 2026 01 04 2026 02 02 Kearns-Sayre Syndrome (KSS) is a rare mitochondrial cytopathy caused by large deletions in mitochondrial DNA, leading to impaired energy production in cells. It has an estimated incidence of 1 to 3 cases per 100,000 population. KSS has no racial or sex predilection. The significant concerns for an anesthesiologist associated with this syndrome are multi-system involvement, including cardiac conduction defects, endocrinopathies, sensorineural deafness, and neurological dysfunction. These manifestations make it challenging for the anesthesiologist. These concerns significantly influence the choice of anesthesia given to the patient, particularly regarding the selection of neuromuscular blockers and their effects on patients with this syndrome compared to those in the general population. Anesthetic management of Kearns-Sayre syndrome patients requires careful preoperative assessment of cardiac, neurological, and metabolic status due to risks of heart block, malignant hyperthermia, respiratory muscle weakness, and lactic acidosis. Also, intraoperative and postoperative vigilance is necessary to prevent any adverse events. We report a 48-year-old female, a known case of Kearns-Sayre Syndrome, scheduled for hysteroscopic polypectomy. This case highlights the importance of detailed preoperative evaluation and tailoring the anesthetic technique to minimize mitochondrial stress. https://aacc.tums.ac.ir/index.php/aacc/article/view/1553