: A right atrial myxoma extending to inferior vena cava (IVC) with associated right-to-left shunting leading to systemic desaturation is an exceedingly rare clinical entity. The number of such cases reported in literature till date is not more than five. This case study presents a 45-year lady who was referred to our Centre with symptoms of breathlessness, easy fatigability, generalized weakness and bluish discoloration of lips, fingers and skin. On routine blood investigation, polycythemia was noted. On preoperative 2D-Transthoracic Echocardiography (TTE), the diagnosis of right atrial myxoma was confirmed but the reason for cyanosis was not revealed. Tumor thrombus in a branch of right lower pulmonary artery with small locoregional pulmonary infarct along with right atrial myxoma was reported in CT angiography of heart and great vessels and CT pulmonary angiography. On-table Transesophageal Echocardiography (TEE) showed patent foramen ovale (PFO) with right-to-left shunting because of raised right atrial pressure and dynamic obstruction of the tricuspid valve by the myxoma. The myxoma was resected completely via right atrial approach along with primary closure of PFO. The postoperative period was uneventful and the saturation at room air of the patient was 100% and she was discharged on day 7. The Patient is doing well during the 3-month follow-up.