Renal Angiomyolipoma occurs both sporadically and in association with tuberous sclerosis [1]. Earlier regarded as a hamartoma, angiomyolipoma is now believed to be neoplastic, showing clonal non random inactivation of the X chromosome [2]. Though generally regarded as benign, angiomyolipomas rarely produce complicated clinical courses that can be life threatening. It is a common cause of severe perirenal and retroperitoneal haemorrhage [3].More rarely they display malignant potential with rapid growth and metastasis. These malignancies associated with angiomyolipoma include malignant epithelioid angiomyolipomas and sarcomatous transformation of one of the components of the tumor, most commonly leiomyosarcoma [3–6]. Extrarenal growth and vascular invasion are rare [7].