Leber's hereditary optic neuropathy (LHON) is a genetic disease featuring maternal inheritance. mtDNA m.11778G>A, m.14484T>C and m.3460G>A mutations are its main molecular basis, but the disease is also affected by nuclear genes, genetic background of mtDNA and environmental factors. By referring to basic and clinical research in the related fields at home and abroad, guidelines and consensus issued by other countries, and combined with data from the Chinese population, this guideline aims to summarize the genetics knowledge and clinical treatment of LHON, with an aim to improve the clinical diagnosis and standardize the clinical management of patients.