Abstract: Solitary fibrous tumor (SFT) is a spindle cell tumor of mesenchymal origin that typically occur in pleura tissue. Ocular SFT as a rare disease, which may occur in a variety of areas, including eyelid, intro-orbital, intra-bulbar. Ocular SFT is mainly seen in adults，monocular onset. The clinical features of the patients often present with eyelid swelling, proptosis, vision disturbances and a palpable mass. CT and MRI revealed a well-circumscribed ovoid mass with intensely enhancement, because of the prominent blood vascular channels within the tumor. The classic histopathological feature of SFT is ovoid to spindle shaped cell growing in a haphazard manner. Background stroma was fibro-collagenous and richly “storiform pattern and staghorn” vessels. A diffuse and intense staining of CD34 and STAT6 are important diagnostic clue favoring SFT. CD99, vimentin and Bcl-2 are also frequently positive in SFT. Scientists have recently identified NAB2-STAT6 gene fusions in the vast majority of SFT, which may promote the formation of SFT. Radical surgical resection seemed to be the most important treatment segment. When the lesion is not completely cleared or in recurrent and malignant cases, postoperative radiotherapy can be used as an adjuvant therapy. SFT as an intermediate tumor, its biological behavior is between benign and malignant. Because of the possibility of its local invasion, local recurrence or distant metastasis, long-term follow-up is mandatory.