Inflammatory myofibroblastic tumors of the uterus (uIMT) are rare and difficult to diagnose neoplasms, since the morphological characteristics of this tumor are not specific and are found in other pathological changes. In addition, until recently, specific uIMT markers have not been identified and their diagnostic standards not defined. However, in recent years, there have been more and more studies aimed to identify characteristic morphological, immunohistochemical, and molecular genetic features for the differential diagnosis of uIMT. Recent papers studying uIMT indicate anaplastic lymphoma kinase (ALK) as a potentially reliable marker of uIMT. This communication describes a clinical case of uIMT in a 40-year-old woman who has been preoperatively diagnosed with a large subserous interstitial myomatous nodule. The final diagnosis was made, by analysing a combination of morphological and immunohistochemical signs. This clinical case with a literature review is indicated to consider ALK as a key criterion in the diagnosis of uIMT, as well as the relationship between subsequent treatment and the presence of ALK in the studied tissues.