Epithelioid Hemangioendothelioma of Cavernous Sinus

Epithelioid hemangioendothelioma is an uncommon vascular neoplasm. It is rare in the intracranial location. Its occurrence in the region of cavernous sinus is reported only once. Here, we report a case of 49-year-old man who presented with a headache, pain in the right eye, diplopia, and ptosis due to third and sixth cranial nerve involvement. Imaging showed an enhancing lesion in the region of cavernous sinus on the right side. Pterional craniotomy, combined extradural and intradural approach, and subtotal excision of the tumor was done. Histopathology and immunohistochemistry were suggestive of epithelioid hemangioendothelioma which is a rare tumor in this location. We report this case because of its rarity and uncommon location.