Masses occupying the retrolabyrinthine space are uncommon and most often represent paragangliomas, choroid plexus papilloma, endolymphatic sac tumors (ELSTs) or distant metastasis. ELSTs are rare, locally invasive tumors of the inner ear, with less than 300 cases reported in the literature. ELSTs are associated with von-Hippel Lindau (VHL) disease. Patients diagnosed with an ELST should undergo genetic testing for VHL disease, and individuals with confirmed VHL disease should be screened for ELST(s).