Cardiac paraganglioma is an extremely rare clinical en-tities of the heart. The usual investigations for pheochro-mocytoma are necessary, however the most useful, spe-cific to primary cardiac tumors, are coronary angiogra-phy and echocardiography. While complete surgical ex-cision is the standard treatment of mainstay. We report a case of a 55-year-old woman with cardiac paraganglioma located in fight atriaoventricular groove of the heart. As we know, this is the first case that cardiac paraganglioma located in fight atriaoventricular groove of the heart in English literature.