In this article we review renal angiomyolipomas (AMLs) from cases in the world literature and 60 cases from our own institution. We also analyze the role and the place of the various imaging techniques in the detection and diagnosis of AMLs. Discussion is focused on the natural history of this benign neoplasm as an isolated form or as a part of the tuberous sclerosis (TS) complex or as part of a third less certain group of multiple AML without stigmata of TS. We also consider the growth patterns and hemorrhagic risks of AML to help define an approach to the current management of this lesion.