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Objective: To investigate the clinical features of Tolosa-Hunt syndrome (THS), a type of painful ophthalmoplegia. Methods: This was a retrospective case series study. The clinical data of patients diagnosed with painful ophthalmoplegia in the Department of Neurology of Tianjin Medical University General Hospital from January 2019 to December 2022 were continuously collected. THS and other types of painful ophthalmoplegia were diagnosed according to the criteria of the International Classification of Headache Disorders, 3rd Edition. The first symptoms, ocular manifestations, imaging and laboratory findings, treatment (glucocorticoids in THS patients) and outcomes were recorded and compared between patients with THS and the other patients. Results: A total of 35 patients with painful ophthalmoplegia who met the criteria were included. Eighteen patients were diagnosed with THS, including 11 males and 7 females, with an average age of (59.9±13.2) years old (range, 28 to 80 years old). Seventeen patients presented with pain as the initial symptom, 17 patients had ptosis, and 10 patients had abnormal signal lesions in the orbital and cavernous sinus area on MRI on the affected side. Nine patients had abnormal serum immunological results (3 had elevated erythrocyte sedimentation rate and C-reactive protein levels, 6 had positive antinuclear antibodies, and 1 was diagnosed with systemic lupus erythematosus and rheumatoid arthritis). Among the 17 patients with other types of painful ophthalmoplegia, 12 had concurrent diabetes mellitus (including 1 with positive syphilis antibodies and 1 with active pulmonary tuberculosis), 2 had intracranial aneurysms, 1 had ischemic oculomotor nerve palsy, 1 had intracranial infection, and 1 had basal meningitis. Five THS patients experienced symptom relief after 3 days of corticosteroid treatment, and 13 THS patients experienced relief within 1 to 6 weeks of treatment. During the follow-up period (7 to 47 months), 3 patients with THS relapsed. Conclusions: Most patients with THS have pain as the first symptom, with positive signs of cavernous sinus on MRI and abnormal serum immunologic examination results. Other types of painful ophthalmoplegia usually combine with diabetes mellitus or other systemic diseases, which is helpful in the differential diagnosis.