Angiomyolipomas (AML), also known as hamartomas, are benign mesenchymal tumors of the kidneys which consist of vascular tissue, smooth muscles, and adipose tissue, with a higher prevalence in females than males. AML may be associated with tuberous sclerosis, and the growth of the mass may present as hematuria or flank pain. We present a case of a 14-year-old female patient who had a known case of tuberous sclerosis since early childhood. She has a history of numerous bilateral renal masses radiographically consistent with AML.A special and unique entity of our case is the age of presentation which is 14 years and the presence of TSC. In contrast to our case, which was invading the right renal vein and IVC at a young age, AML is well known for its benign nature. According to a recent literature review, the youngest patient reported was 16 years old. Typically, non-complicated AMLs less than 4 cm in size are managed by annual radiological imaging which is preferably a CT scan, while larger AMLs of more than 4 cm that present with perinephric hemorrhages or intralesional aneurysms are treated by partial nephrectomy or selective angioembolization.A radical nephrectomy and IVC thrombectomy are typically necessary due to the risks that the IVC thrombus carries as well as the AML itself and its unpredictable behavior. In cases like ours with the extension into the renal vein and IVC, the surgical approach is similar to the venous invasion of renal cell carcinomas.