We present a rare case of a patient with giant right atrial myxoma and simultaneous pulmonary embolism, with good results after surgical removal of the tumour. A 62-year-old woman was admitted to the hospital due to fatigue increasing for several weeks before admission. She complained of progressive exertional dyspnoea and had a medical history of three-months anaemia. On admission she was in a good general condition without clinical signs of heart failure. Transthoracic and transoesophageal echocardiography revealed a mobile mass about 7 cm in length attached to the posterior wall of the right atrium (Figs. 1–3). It prolapsed through the tricuspid valve into the right ventricular outflow tract during diastole. Examinations did not reveal any pathological structures in the main veins draining into the right atrium. Computer tomography (CT) of the chest showed a solid, heterogeneously-hypodense mass, poorly delimited in the right atrium and ventricle, and with dimensions 80 × 45 mm. The lower limb compression venous Doppler ultrasonography showed no signs of thrombosis. Moreover, the pulmonary embolism was confirmed by angio-CT. It revealed the multiple emboli in lobar and segmental pulmonary arteries. The patient was urgently qualified for cardiac surgery and underwent it without complications. The tumour was completely removed (Fig. 4). Histological examination of the tumour confirmed myxoma. Primary tumours of the heart coexisting with a pulmonary embolism are rare. Right atrial myxoma is observed only in 15–20% of myxoma cases with high potential of pulmonary embolism. Immediate surgical treatment is indicated on account of the high risk of pulmonary embolism or sudden cardiac death.