Fibrillin‐1 is an important constituent of the vascular wall and earlier studies have indicated an effect of the Fibrillin‐1 (FBN1) 2/3 genotype on blood pressure as well as aortic stiffness in men. The aim was to determine if the FBN1 2/3 genotype was associated with presence of carotid plaque and incident cardiovascular morbidity and mortality in middle‐aged subjects. The FBN1 genotype was characterized in 5765 subjects (2424 men, 3341 women; aged 45‐69 years) recruited from the Malmö Diet and Cancer Study Cardiovascular Cohort, Sweden. Plaque occurrence and intima media thickness (IMT) of the carotid artery were assessed by ultrasound. Incidence of first cardiovascular events (myocardial infarction and stroke) and cause‐specific mortality was monitored during a mean of 13.2 years follow‐up. The most common FBN1 genotypes were 2/2, 2/3 and 2/4 which accounted for 92.2% (n=5317) of the subjects. There were no differences between the three genotypes regarding age, blood pressure, glucose, lipids, smoking habits, CCA diameter and IMT in men and women. Presence of plaque in the carotid artery was higher in men with genotype 2/3 as compared to the 2/2 and 2/4 genotypes, (55% vs. 46% and 50%, P=0.007). No similar difference was observed in women. No significant relationship was observed between FBN1 genotypes and incidence of cardio vascular disease or all‐cause mortality. The increased prevalence of plaque in the carotid artery of middle‐aged men with FBN1 2/3 genotype indicates a pathological arterial wall remodelling with a more pronounced atherosclerotic burden. This article is protected by copyright. All rights reserved.