Cerebral small vessel disease （CSVD） is a type of vascular disease that affects the whole brain， and it is often associated with high prevalence rate and disease burden. At present， the etiology and treatment of CSVD remain unclear. As a noninvasive marker， heart rate variability （HRV） is used for the quantitative assessment of autonomic nervous function， and it mirrors the equilibrium of the nervous system and is closely associated with blood pressure regulation， inflammatory response， and metabolic processes. Previous studies have shown that the reduction in HRV may be associated with the development and progression of CSVD， although there is a lack of consistent findings. This article reviews the studies on the association of HRV with the imaging and clinical manifestations of CSVD and further explores the possible pathogenic mechanism of CSVD， in order to provide new directions for the prevention and treatment of CSVD. ［Journal of International Neurology and Neurosurgery, 2024, 51(4): 60-64］