Epithelioid hemangioendothelioma (EHE) of bone is a malignant vascular neoplasm with a very low global prevalence. Nonspecific clinical and histopathological findings make the diagnosis of this tumor very challenging. In this case, we reported a 23-year-old male presented with persistent right buttock pain for 9 months (VAS 2-3). No other clinical findings were found. On plain radiographs, a lytic lesion was found on the right iliac wing. MRI showed iso-hyperintense lesions on both T1- and T2-weighted sequences with hemorrhagic components. A CT guided core needle biopsy was performed for further evaluation. The patient underwent wide excision with adjuvant chemotherapy. At six months post-surgery, lymph node metastases was found on PET scan without any clinical symptoms. A comprehensive interdisciplinary evaluation is required to establish the diagnosis of EHE. © 2025 ARSMB-KVBMG. All rights reserved.