The prevalence of cardiac tumors is 0.021% (1 person in 5000). These data are based on a meta-analysis of pathological studies from the early decades of the twentieth century. It is likely that nowadays the incidence of cardiac tumors and primary tumors of the heart is greater. The cardiac tumors are very rare and histologically heterogeneous. These leads to, the lack of established diagnostic schemes, that would allow to distinguish cardiac tumors from metastatic tumors, intracardiac thrombus, vegetations on the valves in infective endocarditis or in the process of connective tissue diseases or thrombotic changes of the valves in the process of metastatic disease (called endocarditis marantica). Cardiac tumors may be symptomatic or found incidentally during evaluation for a seemingly unrelated problem or physical finding. In symptomatic patients, a mass can virtually always be detected by echocardiography, magnetic resonance imaging (MRI), and/or computed tomography (CT). Because symptoms may mimic other cardiac conditions, the clinical challenge is to consider the possibility of a cardiac tumor so that the appropriate diagnostic tests can be conducted. There are very few studies, assessing the results of cardiac surgery in the individual centers. There is also lack of set of standards in the chemotherapy of malignant cardiac tumors. Moreover its routine use is sometimes discussed. JRCD 2014; 1 (8): 5–10