Rationale:Inflammatory myofibroblastic tumor (IMT) is a rare type of mesenchymal tumor. IMT can arise in multiple anatomic locations. IMT of the lumbar spinal canal is exceptionally rare.Patient concerns:Here, we report the case of a 56-year-old male patient with an IMT who was in good health until 1 year prior to admission, when he began experiencing pain in both lower extremities and the lower back.Interventions:A space-occupying lesion in the lumbar canal was identified by magnetic resonance imaging and then surgically resected.Diagnoses:Histopathological analysis of the lesion revealed a composition of mucous edema, inflammatory cells, collagenous fibers, and spindle cells that were diffuse and positive for smooth muscle actin and CD68; focal positive for vimentin and desmin; and negative for CD34 (marker of vascular endothelial cells), CD21, CD23, CD35, S-100, Epstein–Barr virus infection, Ki-67, and anaplastic lymphoma kinase. Thus, the diagnosis was an IMT of the lumbar canal.Outcomes:In the spinal canal, IMT should be considered in the evaluation of tumors although it is a very rare diagnosis. It is a benign lesion, but it has potential for invasion and recurrence.Lessons:There are no characteristic imaging features of these tumors, but they can be addressed by complete surgical excision. Patients with these lesions should undergo frequent long-term follow-up to detect and address recurrence.