<i>PRCC</i> can fuse with <i>TFE3</i> causing <i>PRCC</i>-<i>TFE3</i> fusion-related RCC, a unique subtype of <i>TFE3</i>-RCC.<h4>Case presentation</h4>We report a case of <i>PRCC</i>-<i>TFE3</i> RCC in a 29-year-old woman who was hospitalized owing to a mass in her upper abdomen. Here, TFE3 is linked to renal cell carcinoma.