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Introduction Leber Hereditary Optic Neuropathy (LHON) gene therapy trials are complicated as the relationship between structural and functional outcomes is unclear. The purpose of this study was to assess the correlation between retinal structure and best corrected visual acuity (BCVA) in LHON.Methods Retrospective review of nine patients with LHON over 2 years. Demographics, BCVA and optical coherence tomography (OCT) of the optic disc and macula were collected. The correlation between central retinal layers (total central retinal thickness (CRT), retinal nerve fibre layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL)) and the peripapillary RNFL (ppRNFL) and BCVA was assessed.Results The mean age of patients was 44.4±17 years (females:males ratio 3:6). Data was available for 8, 6 and 18 eyes in the subacute (<6 months), dynamic (6–12 months) and chronic (>12 months) phases, respectively. Compared to 54 age-matched controls, there was significant thinning of ppRNFL in the subacute phase ( p<0.01) followed by significant thinning in the dynamic (p<0.05) and chronic phases (p<0.01). There was significant thinning in the CRT, RNFL, GCL and IPL across all 3 phases (p<0.05). Correlation analysis showed BCVA (logMAR letters) has significantly positively correlated with ppRNFL (p<0.005) and significantly negatively correlated with CRT (p<0.001), RNFL (p<0.05) and IPL (p<0.05) thicknesses.Discussion Our observations support the analysis of structural biomarkers in LHON studies as they may more objectively account for visual function in these patients. Future studies should include functional assays such as microperimetry and ganglion cell electrophysiology.