A 62-year-old female with a history of anal gland adenocarcinoma presents with metastatic disease to the kidney with renal vein tumor thrombus extending into the inferior vena cava (IVC). Metastatic disease to the kidney with renal vein tumor thrombus is extremely rare with only several cases described in the literature. We present the first reported case of metastatic anal gland adenocarcinoma to the kidney with renal vein tumor thrombus.