A solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm of mesenchymal cell origin that develops from the intrathoracic serous membrane, which lines the inner chest wall, mediastinum, and lungs. SFTP is rare, with a reported incidence of 2.8 per 100,000, and contributes to <5% of pleural-based tumors. The neoplastic cells are considered submesothelial fibroblast-like mesenchymal cells and can arise from the visceral or parietal pleura. The precise pathogenesis is uncertain; however, several genetic mutations have been identified, including a characteristic NAB2-STAT6 fusion gene. Clinically, patients may be asymptomatic or present with a nonspecific cough or dyspnea, reflecting indolent growth of the tumor. Radiologic imaging reveals variable-sized, well-circumscribed, round lesions with soft-tissue attenuation. Histologic evaluation demonstrates elongated spindle cells with eosinophilic intercellular connective tissue arranged haphazardly. Immunohistochemistry shows expression for CD34+, bcl-2, Vimentin, and STAT6. The tumor is more commonly benign but can be malignant in 10–20% of cases due to transformation from a benign lesion or de novo. The malignant potential of these tumors carries an increased mortality due to local invasion, increased recurrence risk, and metastatic potential. In up to 10% of cases, SFTP is associated with paraneoplastic syndromes, Pierre–Marie–Bamberg syndrome, and Doege–Potter syndrome, which result in hypertrophic osteoarthropathy and refractory hypoglycemia, respectively. Curative management of SFTPs relies on complete surgical resection. The risk of recurrence necessitates long-term post-resection surveillance.