The leucine-rich pentatricopeptide repeat (<i>LRPPRC</i>) gene is one of the mitochondrial-related functions genes; variations in these genes are responsible for complex phenotypes that affect many organs such as the brain, liver, and muscles.<h4>Materials and methods</h4>This study enrolled a family with Leigh syndrome-like phenotype. The gene discussed is LRPPRC; the disease is Leigh syndrome.