Objective: We present a rare case of surgical management of giant myxoma of the right heart with obstruction of the orifice of the inferior vena cava (IVC), obstruction of the right atrioventricular orifice, with the development of high pulmonary hypertension, and severe heart failure. Case presentation: A 39 years old male patients applied to the consultative polyclinic with typical complaints of severe shortness of breath, palpitations, severe weakness, almost bed rest, severe swelling of the lower extremities, with a transition to the anterior abdominal wall (edema). On physical examination signs of severe heart failure NYHA class IV were revealed. Imaging demonstrated giant right atrial myxoma obstructing IVC with base originated in fossa ovalis, obstructing tricuspid valve and protruding in the right ventricle occupying 50% of its cavity. The surgical removal of the giant RA myxoma protruding the right ventricle and obstructing IVC and tricuspid valve was performed under cardiopulmonary bypass and hemodynamic support. The patient was discharged on the 10th day after surgery and only residual minimal tricuspid regurgitation was revealed on follow-up examination. Conclusion: The described case from our practice proves the possibility of a severe course of giant myxomas of the right heart, with the development of deep hemodynamic complications of systemic and pulmonary circulation. The chosen correct tactics of surgical treatment provided a favorable result, despite the severity of the initial condition and the degree of hemodynamic disturbance. Intraoperative tactics and technique for performing the stages of urgent surgery were chosen based on the situation, the features of the anatomical structure of the tumor, and the severe complications that arose. Our case proves the possibility of adequate implementation of such interventions, when choosing the right tactics of surgical treatment. Take home message: If clinical signs of circulatory failure, heart failure are detected, it is necessary to perform echocardiography. If mass formations of the heart chambers are detected, immediately refer the patient to specialized cardiac surgery clinics to resolve the issue of surgical correction of pathology.