Abstract. Leigh syndrome is one of the illnesses that is caused by genetic mutation that leads to a defect in mitochondrial energy production and it affects the central nervous system, typically appearing in infancy. It is a heterogeneous disorder and encoded by two genomes: mitochondrial and nuclear. Leigh Syndrome is a rare and serious disease that mostly affects young children. It happens when problems in the mitochondria, the parts of cells that make energy, stop the body from working properly. This article covers information about the history of this disorder and explains what the mitochondria is. Types, features and diagnosis of the leigh syndrome are explained after that.