Renal angiomyolipomas (AMLs) are relatively common tumors containing fatty tissue, blood vessels, and muscular cells in various proportions. Typical AMLs can be diagnosed without histological confirmation by a combination of ultrasound and computed tomography imaging in up to 95% of cases. In contrast, simultaneous involvement of the kidney, renal vein, or lymph nodes is uncommon and might be confused with a metastasizing malignant tumor. We present a pathologically proven case of the very uncommon simultaneous involvement of the kidney and the lymph nodes in AML.