Introduction: Renal cell carcinoma (RCC) reveals a tendency towards venous invasion in its advanced stages, making clinical management challenging. Survival may be improved following surgery, but is less clear if it applies to patients with metastatic disease at diagnosis. Materials and Methods: Review of clinical files of patients submitted to surgery for RCC at our institution. Results: Twenty-one patients underwent radical nephrectomy and thrombectomy from 2000 to 2017, with a median follow-up of 25 months. Eighteen (85.7%) men and 3 (14.3%) women, with median age of 63 at the time of diagnosis, were included. The thrombus was in the renal vein in 10 (47.6%) patients and had extension to inferior vena cava (IVC) in 11 (52.4%). The level of involvement in IVC was grade II in 4 (19%) cases, grade III in 4 (19%) cases and grade IV in 3 (14.4%) cases. Surgery was successful in all but 1 patient. Ten patients died during the follow-up (47.6%), resulting in a 5-year overall survival of 34.8%. When considering the metastatic population at diagnosis, the overall survival at 5 years was 45.7%. Conclusion: Radical nephrectomy with removal of the venous thrombus remains the preferential treatment for patients with RCC with venous thrombus, even if they have metastatic disease at diagnosis.