Primary cardiac sarcomas are very rare and the prognosis is poor with a reported survival of usually less than one year after diagnosis. There is few data available for therapy recommendations and outcome. We present the case of a 16 year old patient with a solitary angiosarcoma situated in the right atrium. A complete resection was followed by chemotherapy. Follow up more than 5 years after the initial diagnosis was uneventful with no signs of tumor recurrence. Epidemiology, diagnosis, treatment, follow up and prognosis of cardiac sarcoma are discussed.