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An unusually large renal angiomyolipoma peeping into the right atrium. Angiomyolipomas (AMLs) are benign hamartomatous tumours of the kidney that occur sporadically or in association with tuberous sclerosis. Although they are benign lesions, larger tumours can, rarely, behave aggressively and have extrarenal extension. Extension of an AML into the renal veins and inferior vena cava (IVC) is rare. We report a rare case of an AML with extension into the renal vein and IVC up to the right atrium that was successfully managed surgically.