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Background/Purpose: This study evaluated the long-term outcomes of nephrectomy and inferior vena cava (IVC) thrombectomy in patients with advanced renal cell carcinoma (RCC). Methods: From January 1996 to December 2013, we retrospectively collected the clinical data of 31 patients with pathologically confirmed RCC and IVC tumor thrombus. All patients received nephrectomy and IVC thrombectomy. We examined the clinical outcomes, including cancer-specific survival, progression-free survival, and postoperative complications, during a 90-day period. The associated prognostic factors were also analyzed. This study is registered with the National Taiwan University Hospital Research Ethics Committee (number 201305059RINC). Results: The median follow-up period was 24 months. Of 20 patients with nonmetastatic disease, 15 developed metastases during the study period. The overall 5-year cancer-specific survival rate was 30.8% in the nonmetastatic group versus 12.5% in the metastatic group. The level of IVC thrombus (Levels I–II vs. Levels III–IV) was not significantly associated with the 5-year cancer-specific survival (P = 0.43). The Fuhrman grade and sarcomatoid type were statistically significant predictors of cancer-specific survival (Fuhrman Grades III–IV vs. Grades I–II: Hazard ratio [HR] = 0.11, P = 0.04; sarcomatoid type: HR = 0.136, P = 0.001). Fuhrman grade, capsular invasion, and positive surgical margins were associated with 1-year progression-free survival (Fuhrman Grades III–IV vs. Grades I–II: HR = 0.08, P = 0.04; capsular invasion: HR = 0.15, P = 0.04; positive surgical margins: HR = 0.16, P = 0.05). The most common perioperative complication was massive blood loss. The most common recurrence sites were bones, liver, and lungs. Conclusion: The pathologic Fuhrman grade, sarcomatoid type, positive surgical margins, and capsular invasion were significant predictors of oncological outcomes in patients with advanced RCC and IVC thrombus.