We describe a case of a rare primary chondrosarcoma of the heart in a 62-year-old woman who was referred to our institution with a diagnosis of left atrial undetermined mass. Despite utilization of transesophageal echocardiography and multislice computed tomography imaging, the definitive diagnosis of chondrosarcoma was achieved only with histology of the operative specimen. The tumor was extending from the left atrium to the left pulmonary veins causing obstruction of the venous drainage and a clinical and radiological picture of persistent respiratory infection. Surgery achieved only partial resection of the mass, including the removal of the obstruction to the pulmonary venous drainage. After surgery, a rapid healing of the respiratory infection was observed; however, 3 months later, the tumor was already regrowing rapidly.