Angiomyolipoma (AML) is a benign renal tumor that is member of the perivascular epithelioid cells tumor group. They have a propensity to grow over time and may cause local complications. Although AMLs are classically associated with the tuberous sclerosis complex, approximately 70% to 80% of renal AMLs occur sporadically. They have a very large clinic spectrum between an incidental asymptomatic small renal lesion and life threatening retroperitoneal bleeding. A small proportion of AMLs can present with an aggressive behavioral pattern. Classical ultrasound finding is a hyperechogenic lesion with acoustic shadow. The demonstration of fat on computed tomography or magnetic resonance imaging within a solid renal lesion is usually confirmatory of the diagnosis. Whereas small lesions can only be kept under surveillance, large lesions, lesions that cause to pain or bleeding or lesions that can not be differentiated from RCC may require intervention. Given the benign nature of these lesions, renal-preserving treatments are favored.