Background <p>IgG4-related spinal pachymeningitis (IgG4-RSP) is rare, particularly in cases where spinal cord cavities develop after the removal of thickened dura mater, making diagnosis challenging. A 31-year-old male presented with IgG4-RSP, and the clinical presentation, diagnostic imaging, pathology, treatment, and follow-up outcomes were documented. We also conducted a thorough review of the relevant literature.</p> Case report <p>The patient presented with two-year progressive lower limb weakness. He experienced lower back pain, numbness in both legs, and urinary and defecatory difficulties without a notable medical history. Initial magnetic resonance imaging (MRI) revealed dura mater lesions and thoracic spinal cord compression, leading to surgery that did not improve the symptoms. Pathological examination confirmed IgG4-related hypertrophic pachymeningitis. Physical examination revealed a reduced pain sensation below both rib margins and a muscle strength score of 3/5 in the lower limbs. Thoracic MRI revealed significantly enlarged spinal cord cavities compared to previous scans. Methylprednisolone treatment partially improved symptoms. Subsequent efgartigimod alfa treatment did not alleviate the symptoms further. The patient exhibited significant neurological deficits.</p> Literature review <p>Studies were identified through PubMed, resulting in 20 case reports that met the criteria and were pathologically confirmed. More than half of all patients with IgG4-RSP experience neck or back pain, with lesions typically found in the cervical spine and extending to the thoracic level.</p> Conclusions <p>Caution should be exercised in the post-surgical spinal cord cavity. One-quarter of patients have a poor prognosis. Further research is needed to understand the pathophysiology of IgG4-RSP and develop specific treatments.</p>