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This paper reports the case of 34-year-old woman who presented with bilateral renal angiomyolipomas (AMLs). On the right side, there was a large AML with a fatty thrombus extending to the right atrium. The treatment consisted of right nephrectomy and complete thrombectomy with extracorporeal circulation and right atriotomy. Postoperatively, the patient was septic and died on postoperative day 7 because of septic shock.

Renal Angiomyolipoma with Fatty Thrombus Extending to the Right Atrium: An Exceptional Presentation