There are only limited reports on the voice and swallowing manifestations in children with mitochondrial disorders. This report describes the same in two children with mitochondrial disorders. The first patient with mitochondrial encephalopathy ragged red fiber syndrome had baseline speech-language delay, cognitive dysfunction, and dysarthria. The second patient with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome had prominent swallowing dysfunction and dysarthria. Recognition of otolaryngological manifestations in children with mitochondrial disorders is important to enable better care of these patients.