Inflammatory myofibroblastic tumors (IMTs) mainly occur in children and young adults, usually in the first two decades of life. IMT-type tumors belong to neoplasms of an intermediate biologic potential with considerable rate of local recurrence and in some cases that able to create metastases. Presented case is the first IMT coexisting with the other neoplasm. In our paper we are going to present a peculiar case of an IMT of the bladder coexisting with an ovarian teratoma, and to discuss its pathogenesis, histological picture and differential diagnosis. A 19-year-old female was admitted to the Gynecological Department and during the surgery, two independent, non-adjacent tumors were found. To settle the diagnosis, a FISH examination with the ALK1 break apart probe was carried out. It confirmed the rearrangement of the chromosome 2p23. Morphologic and immunophenotypic similarities between an IMT and other malignant tumors of the bladder may lead to diagnostic errors and an unnecessary radical cystectomy as a result. The therapy of choice is only total excision of the tumor. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1937487606122622