We describe here a 42-year-old woman who was admitted to hospital with a pedunculated mass in her left atrium. She was diagnosed with a primary cardiac osteosarcoma with special immunohistochemical characteristics. Echocardiography and computed tomography can be used to differentiate cardiac osteosarcomas from routine intracardiac tumors. The patient was treated by surgical removal of the mass. Two years later, she has shown no evidence of disease recurrence. We discuss primary osteosarcomas in the cardiac cavity and their management.