Endolymphatic sac papillary tumor (ELST) is an extremely rare and aggressive tumor characterized by hearing loss and temporal bone destruction. A case with clinical, imaging, pathologic and treatment data is reported and relevant literature is reviewed. A 25-year-old woman, with ELST underwent craniotomy for tumor subtotal resection, and the diagnosis was confirmed by pathologic examination. Postoperative radiotherapy consisted of 50.4 Gy/28 f was given accordingly. The patient is currently alive with no signs of tumor recurrence locally and no radiation side-effects observed after one year follow-up. Complete resection is impossible in most cases, local resection, adjuvant radiotherapy may provide favored local control. A long-term follow-up is highly advocated in consideration of its slow development course.