The majority of cardiac myxoma occur sporadically as isolated lesions in the left atrium of middle-aged women. However, a familial form and a syndrome form of this lesion have also been identified. The syndrome myxoma can present itself with pigmented skin lesions and peripheral or endocrine neoplasms. The familial and syndrome forms of cardiac myxomas can usually be distinguished from the sporadic form by their occurrences at younger ages, their unusual locations, the multicentricity of the lesions, and the presence of rare pathological conditions. In addition, a higher rate of recurrent lesions is usually associated with the familial and syndrome forms of this disease. We present a case of complex cardiac myxoma with pigmented skin lesions and breast myxoma and report it with a review of literature.