Primary cardiac sarcomas are rare clinical entities with an incidence rate of 0.0001% in collected autopsy series and are regarded as very aggressive tumors. We herein describe a 21-year-old woman presenting with syncope, dyspnea, and abdominal distention. She suffered from massive ascites, plural effusion, and liver congestion demonstrated by abdominal sonography and chest X-ray. Transthoracic echocardiography revealed a heterogeneous solid mass located in the right atrium; therefore, the patient underwent radical surgical excision of the tumor and 3 cycles of adjuvant chemotherapy. Fifteen months after surgery, she was having a favorable life quality without any evidence of recurrence.