Background. Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm of intermediate malignant potential, commonly arising in the lungs and intra-abdominal organs. Involvement of the urinary bladder is exceptionally rare, particularly in children, and may clinically and radiologically mimic malignant tumors.Case Presentation. We report the case of a 10-year-old girl who presented with painless macroscopic hematuria and syncope, necessitating blood transfusion. Initial imaging revealed a bladder mass, and biopsy initially suggested rhabdomyosarcoma. Definitive histopathological evaluation, however, confirmed IMT. Partial cystectomy was performed, but due to positive surgical margins and recurrent hematuria, targeted therapy with crizotinib was initiated based on anaplastic lymphoma kinase (ALK) positivity. At 12-month follow-up, the patient remained symptom-free with no evidence of recurrence on imaging.Conclusion. Pediatric IMT of the bladder is a rare but important differential diagnosis for bladder masses. Accurate histological diagnosis is essential, as this tumor may mimic malignancy and influence the treatment plan. Complete surgical excision remains the cornerstone of treatment, while targeted therapies such as ALK inhibitors offer valuable options in cases with residual disease or risk of recurrence. This case highlights the importance of a multidisciplinary approach involving surgery, pathology, and oncology. Further pediatric-focused studies are warranted to refine treatment strategies and define long-term outcomes.Keywords: soft tissue neoplasm, inflammatory myofibroblastic tumor, urinary bladder mass, children, crizotinib