A 20-year-old man presented with proptosis, nasal obstruction, vision loss and cavernous sinus syndrome, ongoing for 6years. Imaging and biopsy confirmed a middle skull base epithelioid hemangioendothelioma arising from the left sphenopetroclival region with infratemporal fossa and intracranial-intradural extension into the left temporal lobe. Preoperative embolization of the left internal maxillary artery followed by a combined neurosurgical (front-temporal orbito-zygomatic craniotomy) and otorhinolaryngology (maxillary swing) approach was performed for tumor debulking. Postoperative radiotherapy and maintenance interferon chemotherapy was given to achieve a favorable outcome at 6months follow-up. We describe the pertinent clinical, genetic, radiological and histopathological features, along with the available therapeutic modalities for a primary giant skull base hemangioendothelioma ¹⁾.