A 45-year-old man weighing 50 kg was admitted to intensive cardiac care unit with a complaint of bilateral lower limbs edema and exertional dyspnea. He was in Class 3 functional class. He had not any history of hospitalization. There was also no mentioned family medical history of cardiac disease. He reported worsening of symptoms in the last week before admission. On admission, he was in stable condition and blood pressure was 107/89 mmHg, heart rate was regular and 99 beat per minute, respiratory rate was 26 breath/min, and he was afebrile. Oxygen saturation, at rest, with finger pulse oximeter in index finger was 97%. In the primary physical examination, we found Grade 2+ of lower limbs edema (), and cardiac auscultation revealed soft Grade 2/6 systolic murmur at the both, right and left sternal border without any evidence of diastolic murmur and “tumor plop” in the apex. Laboratory findings shown a mild hypochromic microcytic anemia (hemoglobin = 10.9 g/dl, hematocrit = 34.9%, mean corpuscular volume = 71.96 fl, and mean corpuscular hemoglobin = 22.47 p.g). Chest X-ray (in posteroanterior view) revealed increased cardiothoracic ratio. Aortic arch was normal, main pulmonary artery was seen flat and right descending pulmonary artery was increased (). In electrocardiogram, there was no sign of abnormal conductive pathways. Cardiac rhythm was sinus tachycardia (). The patient referred to echocardiographic study unit; transthoracic echocardiography showed a giant mobile mass in right side of the heart (11.4 × 4.2 cm) attached to the intratrial septum. Inferior vena cava was plethoric and tricuspid valve (TV), function was disturbed by this giant mass. RV function was reduced (Tricuspid annular plane systolic excursion = 0.85 cm) (). The patient was scheduled for open heart surgery. General anesthesia was performed to median sternotomy. Mild hypothermia strategy (33.0° C) was stablished under cardiopulmonary bypass. During an anoxic arrest, single aortic cross-clamping performed, and then, the tumor was completely excised through a right longitudinal atriotomy. The mass referred to histopathological analysis. Based on the microscopic evaluation, myxoid matrix rich in mucopolysaccharides, and polygonal cells appeared as a star and nest shape without atypical features, compatible with non-malignant myxoma. During cardiac surgery and after tumor removal, saline test was performed by cardiac surgeon. According to this test, the surgeon concluded that inappropriate function of TV was due to mass. After 1 month of discharge, our patient readmitted to the cardiac care unit complaining of palpitations. In electrocardiogram, cardiac rhythm was atrial flutter (). Echocardiography of patient, as shown in, indicated that RV and RA, were severely dilated RV systolic function was severely impaired. TV, had leaflet malcoaptation (2 cm), with severe free low-pressure regurgitation [tricuspid regurgitation (TR)], and mild pulmonary insufficiency. Because of hemodynamic instability, cardiac rhythm converted to normal sinus rhythm by synchronized shock. Because of severe low-pressure TR, the patient was candidate for TV repair (TVR), TVR surgery postponed to 6 months after myxoma excision. In this time, the patient has been closely monitored and controlled by medication.