Background: Inflammatory myofibroblastic tumor (IMT) is a neoplasm of unknown etiology occurring at various sites. By definition, it is composed of spindle cells (myofibroblasts) with variable inflammatory component, hence the name is IMT.
Case presentation: The present case is of a 46 years old woman presented with a history of flank pain, abdominal mass and intermittent hematuria for last 6 months. The initial diagnosis was kept as renal cell carcinoma. Finally, it turned out to be a case of retroperitoneal IMT. The patient was managed by complete surgical resection of the tumor.
Conclusion: IMT is a rare neoplasm of uncertain biological potential. Complete surgical resection remains the mainstay of the treatment.