Background. Tolosa-Hunt syndrome (THS) is an uncommon disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. The disease is characterized by periorbital pain, paresis of the bulbomotor and quick response to steroid treatment. The orbital process may lead to optic nerve atrophy. According to the International Headache Society Classification of 2004, the diagnostic protocol includes magnetic resonance imaging (MRI) and biopsy. Case reports. We presented 46-year old male patient, with THS. The patient had unilateral periorbital pain, inflammatory process in the cavernous sinus, the apex of the orbit and the paranasal sinuses. Inflammatory process had spread into the fascia of the bulbomotor and performed compression to the optic nerve, causing paresis of the bulbomotor, protrusion of the eyeball and atrophy of the optic nerve. Pulse doses of corticosteroids were effective. Regarding the presented patient, diagnostic dilemmas arose from nonspecific sinusitis. The initial ophthalmological diagnosis, based on periorbital pain, drop in visual acuity and the narrow chamber angle was angular glaucoma, which resulted in a delayed diagnosis of THS and the beginning of the treatment. MRI and positive response to the treatment with corticosteroids were relevant for making the diagnosis. Conclusion. According to the International Headache Society Classification of 2004, THS is an entity that occurs rarely, its etiopathogenesis is unknown, it is manifested clinically by unilateral orbital pain associated with simple or multiple oculomotor paralyses, which resolves spontaneously but may recur. MRI orbital phlebography and biopsy are the recommended methods for making diagniosis. In our patient MRI findings and positive response to the corticosteroide treatment were relevant for making the diagnosis.