Renal cell carcinoma is an aggressive tumor which is often advanced at the time of diagnosis; its distant spread can take a lymphatic route or, more often, a vascular route (renal vein and inferior vena cava), but tumor thrombosis of left portal vein extending into the round ligament (the umbilical vein) coming from this tumor, to our knowledge, has never been described. We report the case of a metastatic thrombosis of left portal vein and of the umbilical vein from renal cell carcinoma, developed 13 years after nephrectomy, which was successfully treated with left hepatectomy. The experience with hepatectomy for metastatic renal cell carcinoma has been rarely reported. Nevertheless radical hepatectomy is considered to be the only opportunity for curative treatment for selected patients with metastatic renal cell carcinoma.