Background <p>TFE3-rearranged renal cell carcinoma (TFE3-rRCC) is a rare subtype of RCC. This study aims to compare the clinical characteristics and survival outcomes with clear cell RCC (ccRCC) and explore the feasibility of partial nephrectomy (PN) in early-stage TFE3-rRCC patients.</p> Materials and Patients <p>This retrospective cohort study included 62 patients with TFE3-rRCC who underwent surgical resection at Peking University First Hospital between 2016 and 2024. Propensity score matching (PSM) was applied to match TFE3-rRCC patients with ccRCC patients. Clinical characteristics and survival outcomes were compared between the two groups.</p> Results <p>The median age of TFE3-rRCC patients at diagnosis was 33.0&#xa0;years, with a female predominance (61.3%). The majority of patients were asymptomatic (61.3%). Before PSM, TFE3-rRCC patients were significantly younger and had a higher proportion of pT &gt; 2 tumors compared to ccRCC patients (P &lt; 0.01). After 1:2 PSM, survival outcomes in terms of progression-free survival (PFS) and overall survival (OS) were comparable between TFE3-rRCC and ccRCC in early-stage tumors. In patients with T &gt; 2 tumors, a trend toward worse PFS was observed in the TFE3-rRCC group, though it did not reach statistical significance (P = 0.08). No significant differences in PFS and OS were observed between TFE3-rRCC and ccRCC for patients undergoing PN.</p> Conclusion <p>TFE3-rRCC should be stratified for a more accurate prognosis. Early-stage TFE3-rRCC demonstrates survival outcomes similar to those of ccRCC, and partial nephrectomy is a viable treatment approach for these patients.</p>