Spindle cell lesions of the urinary bladder are uncommon tumors, and are most often spindle cell (sarcomatoid) carcinomas, non-neoplastic reactive mesenchymal proliferations, or soft tissue sarcomas. Inflammatory myofibroblastic tumors (IMTs) may also occur in this location. Herein, we report an unusual case of an IMT arising in a previously uninstrumented bladder of a 27-year-old African American female with systemic lupus erythematosus. To our knowledge, IMT has not been reported in the clinical setting of rheumatologic disease.