Endolymphatic sac tumors are rare neoplasms of the temporal bone, histologically benign, but clinically behaves as malignant tumors causing destruction of surrounding tissues. The tumor originates from the posterior surface of petrous part of temporal bone, where endolymphatic sac is situated anatomically. The tumor causes destruction of the posterior surface of the petrous part of the temporal bone, inner ear, mastoid process, etc. Endolymphatic sac tumor can be both sporadically and in patients with von Hippel – Lindau disease. Due to the rarity of this tumor, it is easy to confuse it with other tumors, such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary thyroid carcinoma or papilloma of the vascular plexus. Computed tomography (CT) and magnetic resonance imaging (MRI) are of great diagnostic importance and play an important role in planning treatment tactics. The optimal method of treatment is resection of pathologically altered tissues. In some cases (incomplete resection of tumor, the patients with concomitant diseases or inoperable cases) receive courses of X-ray or radiosurgery. If a tumor is detected in the early stages, the volume of resection can be minimized while preserving hearing and vestibular function of the inner ear. Recurrence usually happens due to difficulty to identify the extension of the tumor. Diagnosis and correct preoperative planning, with embolization if it possible, will facilitate surgery and avoid subtotal tumor resection due to intraoperative bleeding. Long follow-up period is important in order to avoid recurrences. Insufficient coverage of this problem in the literature is associated with a low percentage of its occurrence in otosurgical practice, which complicates the timely diagnosis and treatment of this type of tumors of the temporal bone, worsens the prognosis.