Renal angiomyolipomas are innocuous benign tumours which rarely behave aggressively. This is a case of a 48 year old Malay lady presenting with right sided abdominal pain associated with a large right sided abdominal mass. She was diagnosed with renal angiomyolipoma of the right kidney complicated by inferior vena caval tumour thrombosis. She successfully underwent a radical nephrectomy and inferior vena caval thrombectomy using cardiopulmonary bypass and deep hypothermic circulatory arrest.