The authors report the case of a 16-month-old male with suspected Leigh disease, which was diagnosed on the basis of the clinical manifestations, abnormal lactate stimulation test, proton magnetic resonance spectroscopy, and neuroradiologic findings. Progressive stridor resulting from bilateral vocal cord paralysis and hypoventilation was evident. The authors suggest that for infants or children who exhibit vocal cord paralysis, mitochondrial disorders, such as Leigh disease, should be considered.