BackgroundSyphilis can mimic the clinical features of many neurological disorders and cause ocular motor cranial nerve palsy (OMCNP). This study aims to characterize the clinical features of neurosyphilis patients presenting with OMCNP. Methods In a retrospective study and literature review, demographic data, clinical presentations, laboratory findings, radiological findings, treatment approaches, and outcomes were recorded and analyzed. ResultsOverall, 0.74 % and 1.64 % of patients presenting to two institutes exhibited syphilitic OMCNP, respectively. A total of 32 patients (23 from the literature) were recruited, with a median age of 46 years and a predominance of males (81.3 %). Meningovascular neurosyphilis was diagnosed in 29 patients (90.6 %), and tabes dorsalis was found in 3 patients (9.4 %). Unilateral OMCNP was present in 24 patients (65.5 %). Unilateral sixth nerve palsy was observed in 10 patients (34.5 %), followed by unilateral third nerve palsy in 9 patients (31.0 %), and unilateral multiple OMCNP in 5 patients (17.2 %). A total of 12 patients (41.4 %) presented with isolated OMCNP, and 4 of these individuals developed other neurological deficits during the disease. Pupillary involvement was found in all patients who presented unilateral third nerve palsies. After treatment, 17 patients (58.6 %) achieved complete recovery of ocular motility; 6 patients (20.7 %) achieved partial recovery; 5 patients (17.2 %) remained stable; and 1 patient (3.5 %) became progressive. ConclusionsPatients presenting with OMCNP alone or combined with other cranial nerve deficits should be screened for neurosyphilis, and the standard treatment regimen should be initiated if a diagnosis of neurosyphilis is established.