To determine functional and oncological outcomes of nephron sparing surgery (NSS) for renal cell carcinoma (RCC). We identified from our kidney database 103 consecutive patients undergoing NSS for solid renal tumors in a solitary kidney. After excluding 17 patients (16.5%) undergoing NSS with palliative intent in presence of preoperatively diagnosed metastatic disease (n = 15) or positive lymph nodes (n = 2) and 6 patients (5.8%) who turned out to have benign tumors, the remaining 80 patients with RCC were analyzed. Mean follow-up is 8.0 years (range: 0.1-25.8). Mean tumor size was 4.2 cm (range 1.2-11 cm). Chronic renal failure requiring hemodialysis developed after NSS in nine patients (11.2%). In the remainder, serum creatinine was 1.72 mg/dl (range: 0.45-4.6 mg/dl) at latest follow-up. The cancer specific survival rates at 1, 5 and 10 years were 97.2, 89.6 and 76%, respectively. The estimated local recurrence free survival rates at 1, 5 and 10 years were 97.8, 89.4 and 79.9%, respectively. Univariate analysis of correlation between clinical and pathologic features with death from RCC showed significant associations for grading and tumor size. The long-term data of our series support the concept of organ-sparing surgery for RCC in a solitary kidney since it provides excellent local tumor control and cancer specific survival and preserves renal function renal function so that 89% of patients remained free of dialysis in the long-run.