The two most common forms of inherited optic neuropathy are autosomal dominant optic atrophy (DOA) secondary to pathogenic variants within OPA1 (OMIM: 165500), and maternally inherited Leber hereditary optic neuropathy (LHON) (OMIM: 535000) [3]. The gene discussed is OPA1; the disease is Leber hereditary optic neuropathy.