Tolosa-Hunt syndrome is a rare self-limiting disease characterized by a painful, unilateral ophthalmoplegia caused by an idiopathic granulomatous inflammation in cavernous sinus, the superior orbital fissure or the orbital apex. We report a case of a 51-year-old man who presented at the emergency department with periocular pain and diplopia. Physical examination, and radiological and laboratory testing performed at that time were diagnostically inconclusive. After hospitalization, the patient was diagnosed with Tolosa-Hunt syndrome. Treatment with high dose steroid was started; by 2 days later, the periocular pain was relieved and other symptoms had improved. The rarity of Tolosa-Hunt syndrome calls for knowledge of the disease and attention by an emergency physician. The case report includes as summary of the syndrome, its` diagnosis and treatment.