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Hypertension is associated with cognitive deficits, probably because it is a major risk factor for the development of white matter hyperintensities (WMH), lacunes, and cerebral microbleeds, which are MRI markers of cerebral small vessel disease. Studies into associations between presence or progression of these MRI markers and cognitive decline in hypertensive patients are rare. We investigated the association of baseline presence and progression of MRI markers of cerebral small vessel disease with cognitive decline over 4 years in patients with hypertension.In this longitudinal study, hypertensive patients underwent neuropsychological assessments and brain MRI at baseline and after 4 years. Presence and progression of periventricular and subcortical WMH, lacunes, and cerebral microbleeds were visually rated.In total, 128 hypertensive patients (90 patients with essential hypertension and 38 hypertensive lacunar stroke patients), mean age: 58.6 ± 12.2 years, were included. Progression of periventricular WMH was associated with cognitive decline in simple regression analysis (P = 0.001) and in multivariable analysis with correction for baseline WMH presence and potential confounders (P = 0.004). In this multivariable analysis, R2 of progression of periventricular WMH was 5.6%, whereas R2 of baseline presence of periventricular WMH was 0.6%. We did not find significant associations between baseline presence or progression of the other MRI markers and cognitive decline.In patients with hypertension, progression of periventricular WMH over 4 years is associated with cognitive decline, whereas we could not show an association between baseline periventricular WMH and cognitive decline. These results emphasize the importance of preventing progression of WMH in hypertensive patients.