Background: Endolymphatic sac tumor (ELST) is a rare neoplasm that arises from the endolymphatic sac and duct with less than 200 cases reported. Although it has a benign histopathological appearance its clinical behavior can be aggressive with potential involvement of the otic capsule, mastoid cells, petrous bone, and cerebellopontine angle structures. ELST can present sporadically or as part of VHL disease in 15 to 25% of the cases, which is characterized by the multisystemic development of a variety of benign and malignant tumors, especially in the central nervous system. Patients with VHL disease are reported to be younger at diagnosis compared with the sporadic cases with a female to male ratio of 2:1. Complete surgical excision is the mainstay treatment for ELST, radiation therapy remains controversial.