Objective The aim of this paper was to help clinicians and pathologists to improve the understanding of thyroid-like follicular renal cell carcinoma（TLFRCC）and avoid misdiagnosis and erroneous treatments by exploring the clinicopathological characteristics. Methods One case of TLFRCC was studied，including the clinical manifestations，histological characteristics and immunohistochemical staining，and the clinicopathological characteristics of TLFRCC were summarized by reviewing domestic and foreign literatures. Results TLFRCC mostly occured in middle-aged women，especially in the right kidneys.. They showed a solitary nodular mass as well circumscribed with taupe and grey on the cut surface. Hemorrhage and cystic changes could be seen in some areas. Microscopically，most of tumor cells arranged in thyroid follicular pattern in different sizes. The follicule-like structure was filled with eosinophilic colloid-like material. Immunohistochemical staining showed that tumor cells were positive for AE1/AE3 and PAX8，while negative for Tg，TTF-1， CAⅨ，RCC，TFE3，WT-1 and neuroendocrine markers，with low tumor proliferation index. EWSR1-PATZ1 fusion gene mutation was found in some tumors. The prognoses of most patients were good，but a few patients died from tumor progression. Conclusion TLFRCC is a rare renal epithelial tumor with low-grade malignancy. It looks not special grossly，but similar to thyroid follicles in histomorphology. Tumor cells have renal tubular epithelial phenotype and special gene mutation， which can assist in accurate diagnosis and differential diagnosis of tumors.