The entity which has come to be known as an endolymphatic sac tumor (ELST) has, in the past, been known as adenocarcinoma of endolymphatic sac origin, aggressive papillary tumor of the temporal bone and Heffner's tumor. ELSTs arise in the vicinity of the inner ear and may extend to involve both the posterior fossa as well as the middle ear and the external ear canal, which may complicate the differential diagnosis ELSTs are typically seen in adults, with only rare descriptions in pediatric patients. They may be sporadic tumors or they may arise as part of the symptom complex of von Hippel-Lindau disease. Clinical signs at presentation range from a mass in the external ear canal to sensorineural deafness to cranial nerve palsies. Imaging studies reveal a destructive lesion of the petrous bone which is heterogeneous on MR scanning. Light microscopy reveals two chief patterns: a follicular pattern, reminiscent of thyroid parenchyma; and a papillary/solid pattern. Both patterns are often admixed in the same tumor, and the individual tumor cells are cytologically bland. Immunohistochemically, ELSTs are typically keratin-, vimentin- and epithelial membrane antigen-positive; they are often S-100 protein-positive and neuron-specific enolase-positive as well. ELSTs are difficult to extirpate surgically (owing to their locally aggressive nature); nevertheless, surgical excision remains the mainstay of current therapy. These are slow-growing (albeit locally aggressive) tumors which have only rarely been reported to metastasize; as such, they remain principally a problem of local control.