A 55-year-old man was transferred to our hospital for removal of cardiac and pulmonary tumors. Transesophageal echocardiography demonstrated a large echogenic mass in the left ventricle. The mass was attached to the posterior wall of the left ventricle and the mitral valve. Chest computed tomography showed a solitary, well-defind nodular lesion in the right upper lung. We performed concomitant resection of cardiac and pulmonary tumors through a midline sternotomy. The pathological diagnosis was cardiac chondrosarcoma with pulmonary large cell carcinoma. Postoperatively pelvic computed tomography, bone and gallium scintigrams did not identify any other active lesion, hence the cardiac tumor was considered to be of cardiac origin. He is alive 20 months after the operation and findings from the cardiac and pulmonary examination are unremarkable. Primary cardiac chondrosarcoma is extremely rare, and to our knowledge, only 13 cases have been recorded. We summarize herein these 14 cases.