Then paper describes the case report of a 56-year female patient who has been long treated for dizziness conditions, wrongly attributed to Meniere disease. We accepted the patient for denervation operation on the left labyrinth. The imaging examination made it possible to change the diagnosis for a pyramidal lesion on the left resulting in a diagnosis of tumor of glomus temporale. The preoperative examination revealed that the tumor originated from saccus endolymphaticus and histological examination confirmed it. The authors discuss clinical manifestations, forms, diagnostics and therapy of this exceptionally rare disease, which may pose a serious therapeutic problem if not diagnosed in an early stage.

        Key words: tumor of saccus endolymphaticus.