Epithelioid angiomyolipoma with vascular invasion: An aggressive presentation of an unusual AML variant

Renal angiomyolipoma (AML) is a typically benign renal tumor that is divided into 2 classes, the classical variant and the more aggressive epithelioid variant. It is extremely rare for an AML to exhibit aggressive features such as vascular invasion. We present the case of a 36-year-old female who presented with right lower quadrant pain for 9 months and was found to have an AML with tumor extension into the renal vein and the IVC. Diagnosis was confirmed with histopathology and the patient was treated with a total nephrectomy. The epithelioid subtype of AML is a rare variant that should be considered in the differential of a renal mass with vascular invasion.