Abstract: To review the clinicopathologic features of inflammatory myofibroblastic tumor of the urinary bladder (IMTUB) and to discuss its diagnosis, treatment, and prognosis. Methods: The clinical data, light microscopy, and immunohistochemical results of two IMTUB cases were analyzed. Follow-up of the two patients was also conducted. Results: Partial cystectomy was performed on Case I. Total cystectomy plus pelvic lymphadenectomy was conducted on Case Ⅱ. The post-operative pathological examination indicated that the sections exhibited spindle neoplastic cells and capillary proliferation, accompanied by infiltration of lymphocytes, eosinophil cells, and plasma cells. Immunohistochemical staining of the Case Ⅰ revealed smooth muscle actin ( SMA ) ( + ), Vimentin ( + ), and anaplastic lymphoma kinase ( + ). Immunohistochemical staining of the Case Ⅱ showed SMA ( + ), CD68 ( + ), Vimentin ( + ), chromogranin A ( + ), and anaplastic lymphoma kinase (-). Tumor cells were negative for CK7, S-100, MyoD1, CK19, Actin, and EMA. Signs of recurrence were not found in the two cases. Conclusion: IMTUB is a rare tumor. Final diagnosis of the tumor depends mainly on histopathological examination and immunohistochemical staining. IMTUB should be differentiated from rhabdomyosarcoma and other spindle-cell carcinomas. Partial cystectomy or transurethral resection is the main choice of treatment.