A solitary fibrous tumor (SFT) is a rare spindle cell tumor-derived from mesenchymal cells. It may be linked to the fusion of the NAB2-STAT6 gene caused by 12q chromosome rearrangement. It can occur in the connective tissue of any part of the body; however, it is most common in the pleura. Solitary fibrous tumors of the pleura (SFTP) are a persistent painless mass with slow growth. With the increase of the tumor, there will be corresponding compression symptoms. Pleural effusion is rare, and the cytology of pleural effusion is mostly negative. Occasionally, SFTP can induce paraneoplastic syndrome, distant metastasis, and malignant transformation. Lung function may have mild to moderate restrictive ventilation dysfunction. CT is a crucial method for the clinical diagnosis of SFTP. The histopathological features of SFTP are the coexistence of sparse and dense areas. CD34, CD99, Bcl-2, and vimentin are the most valuable immunohistochemical markers.The positive expression rate of STAT6 in benign SFT was even 100%. Adhesion or unclear boundary with surrounding tissues, pleural effusion or calcification, tumors with a maximum diameter greater than 10 cm, invasive growth, uneven density, metastasis or recurrence, paraneoplastic syndrome, moderate to severe cell heterogeneity, high Ki67 proliferation index, and low STAT6 expression suggest SFTP may be a malignant tumor. Gene analysis on next generation sequencing may help reveal the mutation characteristics of SFTP. Complete tumor resection is the gold standard of SFTP. Resectability is the most important prognostic factor. Age, size, mitosis, and necrosis are considered risk stratification factors for prognosis. Fortunately, 80% of SFTP are benign and have anexcellentprognosis but need long-term follow-up.We report a case of rapidly growing tumor with pleural effusion within 9 months, who was surgically treated and is currently under follow-up. And the literature is reviewed.