Tolosa—Hunt syndrome (THS) is a rare neuro-ophthalmological pathology caused by nonspecific granulomatous inflammation in the area of the cavernous sinus, which leads to painful ocular motility restrictions and ophthalmoplegia. The disease is characterized by lesions of the III, IV, and VI cranial nerves, with possible extension to branches of the V nerve and oculosympathetic fibers. This review analyses current data on this disease, with emphasis on its clinical presentation, diagnostic criteria and treatment methods. Taking into account the complexity of identifying THS, emphasis is made on the need for careful differential diagnosis with other clinically similar conditions, such as brain tumors, vascular aneurysms, inflammatory processes, etc. Timely detection of the syndrome and initiation of appropriate treatment, including the use of glucocorticoid hormones, can help prevent serious complications in the form of vision loss or permanent impairment of ocular motor function.