Abstract:Solitary Fibrous Tumor (SFT) of the central nervous system (CNS) is a rare mesenchymal tumor. Its clinical manifestations and neuroimaging features are often nonspecific and can mimic other intracranial tumors, particularly meningiomas, making accurate preoperative diagnosis challenging. The 2021 World Health Organization (WHO) classification of CNS tumors has merged SFT and hemangiopericytoma (HPC) into a single diagnostic entity designated as "SFT/HPC". Pathogenetically, this tumor is defined by a characteristic NAB2-STAT6 gene fusion, and consequent nuclear immunoreactivity for STAT6 protein serves as a highly specific diagnostic marker.This review comprehensively examines the epidemiology, pathogenesis, clinical presentation, radiological and pathological characteristics, differential diagnosis, and management strategies for CNS SFT. Gross total resection remains the cornerstone of treatment. The decision for postoperative adjuvant therapy should be individualized based on factors such as the WHO grade and the extent of resection. Adjuvant radiotherapy significantly improves local control in patients with high-grade tumors or following subtotal resection. For recurrent or advanced disease, emerging therapies, including anti-angiogenic agents and fibroblast activation protein-alpha (FAP)-targeted radioligand therapy, show promising results.Establishing a multidisciplinary team approach, led by neurosurgery, is crucial for optimizing the precision diagnosis and treatment of SFT and for improving patient outcomes.