This study presents a rare case and demonstrates the feasibility and efficacy of robot-assisted partial nephrectomy (RAPN) in the treatment of localized TFE3-RCC, indicating that with meticulous planning and selection, RAPN could achieve a favorable prognosis in cases of localized TFE3-RCC.<h4>Case presentation</h4>We report a case of TFE3 gene fusion-associated renal cell carcinoma (RCC) in a 28-year-old woman who presented with a history of recurrent left flank pain for over two years, which had intensified over the preceding month. This evidence concerns the gene TFE3 and hereditary clear cell renal cell carcinoma.