Background and Purpose: Cardiac myxoma is a major primary heart tumor which often causes unexpected symptoms or sudden death. This present study was designed to investigate its clinical pathological features and biological behavior. Methods: A retrospective analysis of the clinical pathologic and immunohistochemical features of 66 cases with cardiac myxoma was conducted. Results: In 66 patients with cardiac myxoma, 61 cases had involvement of the left atrium, one case in both the right ventricular and left atria. The female: male ratio was 2.7:1. Patients had symptoms of blood flow obstruction and systemic alterations with performance of arterial embolization. Tumors were spherical, lobulated or irregular in shape, and soft and brittle. Immunohistochemical markers of vimentin and CD34 in tumor cells were positive. Conclusion: Cardiac myxoma always exists in the left atrium and is more common in women, with diverse clinical manifestations and pathomorphism. Although proliferative activity and the recurrence rate are low, in addition to thorough surgical resection, strengthened review is important for young patients.