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Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm characterized by excessive secretion of fibroblast growth factor 23 (FGF23) and renal phosphate wasting, leading to osteomalacia and subsequent pathological fractures. These tumors are typically small and slow-growing, which limits their detection. The diagnostic key lies in the use of functional imaging studies such as gallium-68 DOTA-Tyr³-octreotate (68Ga-DOTATATE) positron emission tomography/computed tomography (PET/CT), along with structural imaging modalities such as magnetic resonance imaging (MRI), for the identification of the primary tumor. Definitive treatment consists of complete surgical resection, which normalizes phosphate levels and resolves the patient's symptoms. We present a diagnostically successful case of a PMT using CT-guided preoperative coil localization before surgical excision, a procedure not previously reported in the literature for these tumors.