Primary bone xanthoma of the inferior orbital rim

Primary bone xanthoma is an extremely rare benign bone tumor histologically characterized by mononuclear macrophage-like cells, abundant foam cells, and multinucleated giant cells. Xanthomas of the bone usually appear as a benign secondary manifestation of some diseases. Therefore, they are denominated primary xanthomas when the other diseases are discarded.We report a peculiar case of primary bone xanthoma of the inferior orbital rim in the absence of hyperlipidemic conditions. Surgical intervention was performed under local anesthesia via a subtarsal 1-cm incision. Histopathological examination confirmed the diagnosis of bone xanthoma. Primary bone xanthomas are extremely rare. Diagnosis of such lesions is challenging and requires both radiographic and histopathological features assessment. A careful work-up and a full lipid profile should be performed to rule out underlying diseases.