Cardiac myxoma is a rare tumor and occurs sporadically as isolated lesions in the left atrium of middle aged women. However, familial occurrence of the neoplasm has been described to comparison of certain clinical and pathologic features among patients who have nonfamilial cardiac myxona with those of patients who have familial cardiac myxoma showed statistically significant differences. Several case reports described that a familial occurrence of this tumor, also that these are associated skin lesions as well as other endocrine tumor. This so called complex of syndrome form of cardiac myxoma and the familial form have characteristics that distinguish them from the sporadic form. Nonfamilial cardiac myxoma was a disorder of middle aged women, usually occurred in the left atrium as a single tumor and had no particular associated conditions. On the other hand, familial cardiac myxoma was a disorder of young men, was less commonly in the left atrium, was often multicentric, and was occasionally associated with unusual or rare conditions and a tendency for recurrence. Therefore, for these patients, we recommand a thorough search for multiple tumors at operation, close postoperative follow-up, and careful screening of family members. Our findings of atrial myxoma in a brother and a sister associated with pigmented skin lesions strongly suggest a tendency to familial occurrence.