Аннотация:The article describes a clinical observation of a 17-year-old patient with an advanced tumor of theendolymphatic sac in the direction of the middle and posterior cranial fossae, the internal auditory meatus, and the cerebellopontine angle. After selective embolization of tumor afferent vessels, a combined transmastoid, translabyrinthine, and extended approach through the middle cranial fossa was used. The facial nerve is preserved along its entire length. The inferior cranial nerves remained intact when the mass was removed from the jugular foramen. Resection of the lower part of the tumor together with the sigmoid sinus, endolymphatic sac, and bulb of the jugular vein was performed after tamponade of the transverse and inferior petrosal sinus.