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A 52 year old man was investigated for fever of unknown origin and a grade II/IV systolic murmur. The ECG and chest radiograph were normal. The patient had normocytic anaemia, moderate thrombocytosis, and elevated C reactive protein (CRP), alanine aminotransferase (ALT), and aspartate aminotransferase (AST). Investigations for collagen vascular disease were negative. An abdominal ultrasound revealed a 3 × 3 cm mass in the anterosuperior aspect of the right kidney. Contrast enhanced abdominal tomography (panel A), and magnetic resonance imaging (panel B), showed an enlarged right kidney with inhomogeneous density, along with a 3 cm low density mass (arrow). Transthoracic (panel C) and transoesophageal (panels D and E) echocardiography disclosed a mobile echo-dense mass (arrow) protruding from the inferior vena cava (IVC) into the right atrium (RA) and through the tricuspid valve (TV) up to the right ventricular (RV) apex, creating tricuspid regurgitation without stenosis. Urgent surgical treatment was decided upon. The operation was done under deep hypothermia with circulatory arrest for 31 minutes. The intracardiac extension of the tumour was approached via longitudinal right atriotomy (panel F) and the whole mass was resected with the kidney, adrenal gland, and ipsilateral lymph nodes (panel G). A patch of autologous pericardium was used for IVC closure. Extracorporeal circulation and patient rewarming started immediately after closure of the atrium. The patient had an uneventful course. Histological examination of the tumour disclosed clear cell carcinoma of the kidney (panel H), with infiltration of the renal vein, whereas the lymph nodes were not involved. One year later the patient remains disease-free.