We report a case of a cardiac myxoma with cartilaginous differentiation. Cardiac myxomas are the most common primary neoplasms of the heart. The cell of origin is thought to be a pluripotential cell called the "myxoma cell," which is capable of differentiating towards various tissues, including endothelial, epithelial, and smooth muscle; however, cartilaginous differentiation is so uncommon that other entities such as metastatic chondrosarcomas must be considered in the differential diagnosis. These entities are discussed along with the radiologic-pathologic correlation.