Objective: To investigate the clinicopathological features of Xp11.2 translocation/TFE3 gene fusion associated renal cell carcinoma (Xp11.2 RCC). Methods: We collected and analyzed the clinicopathological data of 10 patients with Xp11.2 RCC and to follow up and review the relevant literature. Results: Ten patients aged 18 to 76 years old (median age 33 years old). The maximum diameter of tumors was 3.3–10.0 cm. Microscopically, the tumor tissues were mostly were consisted of papillary or pseudopapillary architecture, partially arranged in a tubular or nested pattern. The tumor cells had abundant clear to slightly eosinophilic cytoplasm, dark stained or vacuolar nucleus with clear nucleoli. The tumor stroma was a fine fibrous vascular septum. Psammoma bodies were visible in 3 cases. Immunohistochemically, 10 cases of tumor tissues strongly and diffusely expressed TFE3, Melan-A, p504S and CK, partial positive for Vimentin, CD10, RCC, PAX8 and EMA, negative for CK7 and CD117. The 9 patients were followed up, including 1 death, 1 pulmonary metastasis, and 7 patients without recurrence and metastasis. Conclusion: Xp11.2 RCC is a rare type of renal cell carcinoma with relatively characteristic histological and immunophenotype. Accurate diagnosis is crucial for subsequent clinical treatment.