AIM: To reveal alterations in retinal structure, vessels, and function, and their association with cognitive function and neuroimaging in white matter hyperintensities (WMH). METHODS: This study enlisted WMH and age-matched healthy controls (HC). All participants underwent six different tests: magnetic resonance imaging (MRI) of the brain, the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), fundus photography, optical coherence tomography (OCT), and visual field testing. Visual field can reflect the function of optic nerve and retina. The peripapillary retinal nerve fiber layer (p-RNFL) was analyzed using OCT. Image J software was employed to measure retinal vascular caliber in fundus photographs and to compute the central retinal artery equivalent (CRAE), central retinal venous equivalent (CRVE) and arteriole-to-venule ratio (AVR). RESULTS: A total of 90 WMH patients and 93 HC participants. In comparison with the HC, the WMH group exhibited reduced cognitive function scores (MoCA: P <
0.001
MMSE: P <
0.001), narrower retinal arteries (P <
0.001), smaller AVR (P <
0.001) and thinner p-RNFL thickness (total: P = 0.026
temporal: P = 0.006). About visual field, both univariate and multivariate analysis showed that mean sensitivity decreased, and mean defect increased in WMH group (P <
0.05). Additionally, correlation analysis indicated a positive correlation between CRAE and AVR with MMSE and MoCA score (r = 0.424-0.57, P <
0.001) and a negative correlation with Fazekas score (CRAE: r = -0.515, P <
0.001
AVR: r = -0.554, P <
0.001), and p-RNFL was negatively correlated with Fazekas score (total p-RNFL: r = -0.192, P = 0.009
temporal p-RNFL: r = -0.217, P = 0.003). Notably, no significant correlation was found between cognitive function and p-RNFL. CONCLUSION: WMH group exhibit narrower retinal arteries, smaller arteriole-to-venule ratio, damaged p-RNFL and visual function. These alterations in retinal vessels are associate with both neuroimaging and cognitive function. Our results suggest that retinal imaging could serve as a valuable instrument for evaluating WMH and provides some new approaches to study the characteristic markers of WMH.