Renal cell carcinoma (RCC) is the most prevalent primary renal malignant neoplasm in adults. Most of the cases are usually found incidentally. It is commonly associated with venous thrombosis. We demonstrate a case of a RCC which was associated with an extensive thrombus that reached the upper part of the inferior vena cava (IVC). We also perform a brief literature review about the association between RCC and IVC thrombosis.