Tolosa-Hunt syndrome (THS) is a rare idiopathic granulomatous inflammatory disease of cavernous sinus or superior orbital fissure. Recurrent painful ophthalmoplegia with paresis of the third, fourth or sixth cranial nerves as well as the first and second divisions of the trigeminal nerve are the typical characteristics of THS. We report a 62-year-old male patient presenting with left side headache and painful ophthalmoplegia. He also accompanied by contralateral facial nerve palsy for 30 years without any specific change. Brain MRI showed an enhanced lesion in the left cavernous sinus and canalicular segment of the right facial nerve. He was ultimately diagnosed with THS and we report this rare case of THS with facial nerve palsy with a review of related literature.