BACKGROUND: Spinal syphilitic gumma is an unusual presentation of neurosyphilis, with a limited number of cases reported in the literature. Owing to its extreme rarity, the diagnosis and treatment of spinal syphilitic gumma and the relevant prognosis have not been outlined. CASE DESCRIPTION: A 65-year-old woman with a history of neck, shoulder, and back pain presented with progressive sensorimotor disturbance of the extremities. Serologic tests revealed the presence of syphilis. Magnetic resonance imaging demonstrated an intramedullary nodule at the C5 level. A suspected diagnosis of spinal intramedullary tumor was made, and surgical resection of the nodule was performed. Histologic and immunohistochemical findings revealed a syphilitic gumma. We also conducted a comprehensive review of the published literature in all languages regarding spinal syphilitic gumma. The clinical and radiologic features, laboratory findings, therapeutic strategies, and follow-up data were analyzed. CONCLUSIONS: Spinal syphilitic gumma can occur in the intramedullary region, and clinicians should be aware of this extremely rare entity, as it can be easily mistaken for more common neoplasms occurring at this site. The magnetic resonance imaging appearance of this entity has some suggestive characteristics, and a confident diagnosis depends on the pathology. Surgical resection can help to relieve acute spinal cord compression, and systemic antibiotic treatment for syphilis should be emphasized for long-term control.