Inflammatory myofibroblastic tumor, also called inflammatory pseudotumor, is an uncommon soft tissue lesion of unknown etiology, which is characterized by inflammatory cells and fibrous stroma. Inflammatory myofibroblastic tumor involving the spinal cord is extremely rare and, to date, only 12 cases have been reported in the literature. We report a case in a 25-yearold man presenting with low back pain, numbness below the umbilical region, progressive bilateral lower limb weakness and urinary incontinence. Magnetic resonance imaging showed an intradural extramedullary spinal tumor with spinal cord compression at the T8-T9 level. The tumor was later totally removed and histological examination confirmed a diagnosis of inflammatory myofibroblastic tumor. Six months later, the patient could walk without assistance and completely recovered without any profound neurological deficits. The relevant literature is reviewed.