Cardiovascular complications are uncommon in Behçet's disease, but are frequently the cause of morbidity and mortality. Venous and peripheral arterial involvement have been well documented, but involvement of the proximal aorta has rarely been described. This report details a Chinese patient with Behçet's disease. Diffuse aortitis led to proximal aortic dilatation and severe aortic regurgitation necessitating aortic valve replacement. Histopathology of the aorta revealed features similar to those observed in other systemic diseases with aortic involvement.