Background <p>IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterized by tumefactive lesions, dense infiltration of IgG4-positive plasma cells, and variable organ involvement. Inflammatory pseudotumors associated with IgG4-RD are uncommon, and spinal nerve root involvement is exceedingly rare. Such lesions may closely mimic malignant spinal tumors on imaging, posing significant diagnostic challenges.</p> Case report <p>We report the case of a 55-year-old woman who presented with progressive lower back pain and urinary incontinence. Magnetic resonance imaging revealed a space-occupying lesion in the left L1/2 intervertebral foramen. ^18F-FDG positron emission tomography–computed tomography (PET-CT) demonstrated intense focal hypermetabolism with a markedly elevated maximum standardized uptake value (SUVmax) of 11.95, strongly suggesting a malignant process. The patient underwent surgical decompression and tumor resection. Histopathological examination revealed a fibroinflammatory lesion with dense lymphoplasmacytic infiltration and abundant IgG4-positive plasma cells (45/HPF in hotspots), consistent with an IgG4-related inflammatory pseudotumor, despite normal serum IgG4 levels. </p> Conclusion <p>This case highlights that, despite strong radiological suspicion for malignancy, preoperative biopsy may allow diagnosis of IgG4-RD and enable corticosteroid therapy, potentially avoiding unnecessary radical surgery.</p>