ABSTRACT
Mitochondrial encephalomyopathies are genetic defects affecting the mitochondrial respiratory chain. This case report describes the anesthetic considerations for a patient with mitochondrial disease undergoing renal transplantation. Special risk such as malignant hyperthermia as well as plausible anesthetic technique are addressed. This is the case of a 36 year old female previously diagnosed to have end stage renal disease secondary to chronic glomerulonephritis and mitochondrial disease who presented for renal transplantation. Anesthetic technique was general endotracheal anesthesia under total intravenous anesthesia. To avoid a life threatening sequelae associated with mitochondrial diseases, vigilance towards possible complications was undertaken.