A case report of a 51-year-old man with left renal tumor and level II vena cava tumor thrombus (thrombus extending >2 cm above the renal vein, but below the hepatic veins) in a rare anatomical variant of renal vein. In nonmetastatic patients, aggressive surgical resection is widely accepted as the standard management option, but some doubts about the best practice in these patients are relevant. Surgical approach on those patients is a challenge for the surgeons, and anatomical variants make the procedure even more difficulty. These patients should be referring to a tertiary center because of the potential perioperative complexity.