Budd-Chiari syndrome has been described as a late complication of Behçet's disease. Although the mortality rate associated with Behçet's disease is low, it can escalate in the presence of Budd-Chiari syndrome and may be further complicated by intracardial thrombus formation. It is therefore important to detect and initiate management early in the disease course. The imaging modalities of choice should be minimally invasive as certain procedures may aggravate Behçet's disease by initiating a thrombosis or aggravating an existing one. In Behçet's disease-induced Budd-Chiari syndrome, cardiac investigation is crucial in the work-up in order to identify any cardiac involvement and determine the etiology of intracardial thrombus. Furthermore, the treatment should ultimately focus on controlling the activity of Behçet's disease. We report an unusual case of Behçet's disease presenting with Budd-Chiari syndrome complicated by intracardial thrombus in a young Korean man.