The small number of cases causes difficulty with an accurate diagnosis. Imaging and immunohistochemical (IHC) studies should be performed to distinguish possible primary sites. IHC studies are not yet close to refusing or confirming the diagnosis. Due to the lack of data, there is no consensus on the proper therapeutic strategy. Prognosis and survival appear to depend upon the stage of disease at diagnosis. Therefore, the awareness of such a histopathological kind of cervical cancer is crucial.