Behçet’s disease (BD) is a systemic vasculitis of unknown etiology. It is characterized by recurrent oral and genital ulcers, uveitis, and a number of systemic manifestations. Although the disease is recognized worldwide, its prevalence is highly variable. A detailed review and analysis of the worldwide published reports on BD showed that not only the prevalence of the disease but also its cardiac complications differ according to the geographic distribution of the studied population. With the exception of France, Greece, Spain, and Italy, very few reports and studies regarding BD have been published from the western countries. Cardiovascular complications are an important cause of poor outcome in patients with BD. Over the past few years, many case reports and studies have been published, providing more facts about these complications. For example, peculiar echocardiographic findings in patients with aortic valve regurgitation and intracardiac thrombi secondary to BD were recently described. The role of these findings in the initial diagnosis of the disease, however, remains to be evaluated. On the other hand, some reports present contradictory results, especially concerning the left ventricular diastolic function, pathogenesis of coronary artery disease, and proper management of the cardiac complications in BD. Importantly, management of these complications is based mainly on the discretion of the treating physician due to the absence of large controlled studies and clear guidelines. This approach sometimes creates inconsistent data and allows controversies to persist. The review presented here will discuss some of the facts and controversies related to cardiac complications in BD.