Background <p><i>TFE3</i>-rearranged renal cell carcinoma is a rare malignancy with distinctive morphological and immunological characteristics. Although it can manifest in individuals of any age, its occurrence in adults is exceedingly uncommon, comprising only 1.5% of adult renal cell carcinomas. The disease typically presents with a benign course in pediatric patients, yet in adults, it often progresses rapidly and aggressively.</p> Case presentation <p>Herein, we present a case of a 35-year-old female diagnosed with <i>TFE3</i>-rearranged renal cell carcinoma, who was initially asymptomatic. Imaging studies revealed a solid renal mass in the left kidney without signs of metastasis. Initially misdiagnosed as a vascular smooth muscle lipoma, the patient underwent laparoscopic partial nephrectomy. Subsequent histopathological and molecular analyses validated the diagnosis of <i>TFE3</i>-rearranged renal cell carcinoma. The patient demonstrated favorable short-term outcomes after early laparoscopic intervention, with no evidence of tumor recurrence or metastasis at both the 4-month and 15-month follow-ups.</p> Conclusions <p>This case serves as a valuable resource for oncologists involved in the diagnosis and management of <i>TFE3</i>-rearranged renal cell carcinoma.</p>