Provision of anesthesia for patients with mitochondrial disorders is associated with a unique set of challenges. These disorders are rare, which complicates efforts to develop high quality, evidence-based guidelines to inform the perioperative management of those who suffer from them. Accordingly, case reports remain an important source of information regarding their care. Here we present the case of a 27-year-old female patient with mitochondrial myopathy and a history suggestive of malignant hyperthermia susceptibility who received general anesthesia for 2 consecutive surgeries. The induction agents included fentanyl, ketamine, and methohexital. The maintenance agents were methohexital, sufentanil, and dexmedetomidine.