Epithelioid hemangioendothelioma (EHE) is a rare form of vascular neoplasm that can manifest with various symptoms or be discovered incidentally in asymptomatic patients. In this report, we describe a case of a 56-year-old male who presented with progressive lower limb weakness over four years. The evaluation revealed severe hypophosphatemia, an inappropriately normal fibroblast growth factor 23 C-terminal (cFGF-23) level, and a 30 x 20 mm hypermetabolic right pleural mass, which was subsequently proven to be EHE.
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