Due to surgical advances, cardiac tumors now represent a potentially curable form of heart disease. Primary cardiac tumors are very rare; secondary, metastatic tumors are 10-40 times more frequent. The majority (75%) of primary tumors is benign. Cardiac tumors may produce hemodynamic disturbances, mechanical hemolysis, biochemical effects and constitutional symptoms. According to the anatomic location of the tumor, some special symptoms and physical findings can be grouped with regard to pericardial, myocardial, and endocardial involvement. Several laboratory data (elevated sedimentation rate, anemia, thrombocytopenia etc.) may give additional hints to suggest cardiac involvement in neoplastic disease. Among the diagnostic procedures, two-dimensional echocardiography has become the most important method for non-invasive detection of cardiac tumors. The importance of invasive procedures has declined by the improvement of non-invasive imaging techniques, but pressure measurements within the cardiopulmonary system, left-sided, pulmonary angiography as well as coronary angiography give additional useful and necessary information prior to surgery. Within the spectrum of differential diagnosis, particularly mitral, aortic, or tricupid valve disease, bacterial endocarditis, or autoimmune diseases have to be ruled out. Thus, in the vast majority of cases the correct diagnosis can be made. This is crucial for further management of the patient since the majority of primary cardiac tumors can be successfully treated by surgery.