Cerebral Small Vessel Disease (CSVD) is a widespread brain-affecting condition with significant prevalence and burden. Despite this, its etiology and treatment remain unclear.Heart rate variability (HRV), a non-invasive marker, quantitatively assesses autonomic nervous system function. It mirrors the nervous system's equilibrium and is closely linked to blood pressure regulation, inflammatory responses, and metabolic processes.Prior research suggests that decreased heart rate variability could be linked to the onset and progression of cerebral small vessel disease, though findings are inconsistent.This review summarizes the research on the relationship between heart rate variability and the imaging, and clinical presentations of cerebral small vessel disease, and explores its potential pathogenic mechanisms, aiming to inform novel directions for its prevention and treatment.