Behcet's disease (BD) is a multisystem disease of unknown etiology characterized by chronic relapsing orogenital ulcers, uveitis and systemic involvement including articular, gastrointestinal, cardiopulmonary, neurological and vascular pathology. The incidence and nature of cardiac involvement are not clearly elucidated. Cardiovascular manifestations have been reported in 7-46% of patients and mortality occurs in up to 20% of those patients with marked vascular involvement. Sporadic cases of endocarditis, myocarditis, pericarditis, acute myocardial infarction, aortic aneurysm, ventricular thrombosis, congestive cardiomyopathy and valvular dysfunction have been reported. This review discusses the general aspects of the pathogenic mechanisms and clinical features cardiovascular involvement in BD, and provides the data of cardiovascular involvement in a cohort of Italian BD patients.