Renal angiomyolipomata can exist as an imaging curiosity or represent a life-threatening condition. There are likely over 10 million people world-wide who have a renal angiomyolipomata and approximately one tenth of these people also have tuberous sclerosis complex. The optimum treatment of angiomyolipomata is focused on sparing renal tissue and has included no intervention, both total and partial nephrectomy, and embolization. As basic science investigation into the biology of angiomyolipomata progresses, there is even hope for successful drug therapy. Because these renal lesions can be associated with other organ system dysfunction, a number of medical specialists become involved in the care of affected patients. The purpose of this article is to update the nephrologist on the molecular understanding of renal angiomyolipomata and for the possibilities of pharmacologic therapy in the future.