White matter hyperintensity (WMH) of presumed vascular origin is a common neuroimaging feature of cerebral small vessel disease. WMH regression has attracted increasing attention in recent years. In this review, we systematically summarize the incidence, influencing factors, pathological mechanisms, and clinical implications of WMH regression. The reported incidence of WMH regression varies widely across studies due to differences in study populations and measurement methods. However, neuroimaging evidence suggests that WMH regression has true biological significance rather than a simple measurement error. Many studies suggest that WMH regression has a protective effect on cognitive function. It holds promise as a surrogate imaging outcome for evaluating treatments for cerebral small vessel disease. Future studies are needed to better understand the underlying mechanisms, thus providing new insights into the treatment of cerebral small vessel disease.