Sclerosing stromal tumours of the ovary are benign and tend to occur in young women with lobular structures at low frequencies. Three types of cells, luteinized cells, short spindle myoid cells, and intermediate cells, are found in lobules, which are rich in blood vessels. Currently, immunohistochemistry and fluorescence in situ hybridization are used to detect normal follicles, sclerosing stromal tumours, granulosa cell tumours, and theca fibromas. Our research found the expression of transcription factor enhancer 3 (TFE3) was moderately and strongly positive in the inner thecal cell layer of normal follicles. It was expressed in seven out of eight sclerosing stromal tumours, mainly in luteinized cells, but not in 20 granulosa cell tumours and 1 microcystic stromal tumour. In nine cases of theca cell tumours and theca fibromas, TFE3 was not expressed, except in two cases of weak TFE3 expression. Eight cases of sclerosing stromal tumours were analysed by FISH using a TFE3 separation probe, and the results were negative. In a word, as a nuclear transcription protein, TFE3 was specifically expressed in sclerosing stromal tumours and could serve as a new marker for the diagnosis and differential diagnosis of sclerosing stromal tumours.