Tolosa Hunt syndrome (THS) is a rare neurological syndrome, manifesting as a unilateral headache with painful ophthalmoplegia. THS main symptoms are thought to be derived from the granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbits. Although the facial nerve does not originate from the cavernous, few case reports have observed the involvement of facial nerve, proposing the idea of a diffuse systemic inflammation. We here describe a case of a typical THS in the form of initial presentation, imaging and response to treatment but has atypical features, in the form of extracavernous cranial nerve involvement.