Simple Summary: Extrameningeal solitary fibrous tumors (SFTs) are distinct mesenchymal neoplasms with the propensity for recurrence and the characteristic genetic marker of the NAB2-STAT6 fusion gene. These tumors typically present as slow-growing masses, most commonly in the extremities or trunk. Different clinical behaviors ranging from low to high aggressiveness with the potential for metastasis can be observed. Diagnosis is based mainly on immunohistochemical staging for classic CD34 and STAT6 markers, although it can be perplexed by the similarity to other lesions. Surgical resection remains the primary treatment, and long-term follow-up is obligatory due to the unpredictable nature of SFTs. Further research is emerging to understand the biological behavior and optimal management with efficient treatment of SFTs. Solitary fibrous tumors (SFT) are rare mesenchymal neoplasms that account for less than 2% of all soft tissue masses. In the latest WHO 2020 Classification of Soft Tissue Tumors, extrameningeal SFT was listed as intermediate (rarely metastasizing) or malignant neoplasms. Due to the lack of characteristic clinical features, their diagnosis and treatment remain challenging. The pathogenesis of SFT is often associated with the presence of fusions of the NAB2-STAT6 gene on the 12q13 chromosome. Cytoplasmic CD34 positive staining is considerably characteristic for most SFTs; less frequently, factor XII, vimentin, bcl-2, and CD99 are present. A key factor in the diagnosis is the prevalent nuclear location of STAT6 expression. Radical resection is the mainstay of localized SFTs. In the case of unresectable disease, only radiotherapy or radio-chemotherapy may significantly ensure long-term local control of primary and metastatic lesions. To date, no practical guidelines have been published for the treatment of advanced or metastatic disease. Classical anthracycline-based chemotherapy is applicable. The latest studies suggest that antiangiogenic therapies should be considered after first-line treatment. Other drugs, such as imatinib, figitumumab, axitinib, and eribulin, are also being tested. Definitive radiotherapy appears to be a promising therapeutic modality. Since standards for the treatment of advanced and metastatic diseases are not available, further investigation of novel agents is necessary. [ABSTRACT FROM AUTHOR]