Inflammatory pseudotumor is a rare benign neoplasm. It is common in children and has been reported in various locations throughout the body but rarely in the heart. Behçet's disease is a multisystemic, recurrent, inflammatory disorder. We report a 35-year-old Behçet's disease patient with pseudotumor of the right ventricle and multiple pulmonary emboli. Transthoracic echocardiography showed dilation of right atrium, right ventricle, and a mass in the right ventricle. Multislice computed tomography revealed a large, poorly defined mass infiltrating the groove and multiple pulmonary emboli. Surgery was performed with diagnostic and therapeutic intent. Following sternotomy, we identified a mass in the anterior wall of right ventricle, the outflow tract, and the inflow tract of right ventricle. The histopathologic analysis identified an inflammatory infiltrate composed of lymphocytes, plasma cells, and other inflammatory cells, without mitosis. And there were also some thrombus on top of the mass.
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