Introduction: Usually presenting in infancy, Leigh's syndrome is an inherited condition often manifesting with seizures, ataxia, developmental delay, and dysarthria. The disorder is rare, appearing in approximately 1 in 40,000 live births. Consequently, providing these patients with a suitable plan by which to administer anesthetics remains problematic.
Case presentation: We report a male patient with Leigh's syndrome and a family history suggestive of unknown hypotonia and malignant hyperthermia presenting for dental rehabilitation.
Conclusions: Dexmedetomidine with remifentanil can be used with no complication in this senerio.
Keywords: Anesthesia; Encephalomyelopathy; Leigh’s Syndrome; Malignant Hyperthermia; Mitochondrial Disorders.