BACKGROUND We examined the relationship between arterial hypertension, localized retinal nerve fiber layer defects (RNFLDs), and retinal vascular abnormalities. METHODS The study included 359 patients with arterial hypertension and 331 individuals without hypertension as control subjects. Localized RNFLDs and retinal vascular abnormalities were assessed on fundus photographs. RESULTS After adjusting for blood concentrations of glycosylated hemoglobin (HbA1c), high-density lipoproteins, low-density lipoproteins, and hyperlipidemia in a multivariable analysis, localized RNFLDs were, in addition to arterio-venous nicking and generalized arteriolar narrowing, significantly associated with arterial hypertension. After adjusting for body mass index, waist/hip ratio, blood concentrations of glucose, HbA1c, triglycerides, high-density lipoproteins, low-density lipoproteins, and prevalence of diabetes mellitus and hyperlipidemia, categories of arterial blood pressure (optimal/normal/highly normal; mild, moderate and severe hypertension) were significantly associated with localized RNFLDs, which were present significantly more often in hypertension grades 2 and 3, with odds ratios (ORs) of 10.01 and 6.45, respectively. The corresponding ORs for arterio-venous nicking (hypertension grades 1, 2, and 3: ORs of 2.00, 1.68, and 1.99, respectively) and generalized arteriolar narrowing (hypertension grades 1 and 3; ORs of 4.60 and 4.32, respectively) were lower than those for localized RNFLDs. CONCLUSIONS Localized RNFLDs, in addition to retinal microvascular abnormalities such as focal and generalized arteriolar narrowing, were associated with different grades of arterial hypertension. Ophthalmoscopic examination for localized RNFLDs may be useful for the assessment of the retinal abnormalities associated with arterial hypertension and for the grading of arterial hypertension.