Objective: To increase awareness of the presentation, diagnostic difficulties and management of endolymphatic sac tumours. Case reports: A 79-year-old man with a 6-month history of unilateral hearing loss, tinnitus and vertigo, who was suspected to have an endolymphatic sac tumour on imaging, underwent successful transmastoid-translabyrinthine resection. A 53-year-old man with unilateral hearing loss and pulsatile tinnitus underwent subtotal resection of a suspected paraganglioma, which was identified histologically. Due to interval growth, gamma knife radiosurgery was performed followed by subtotal petrosectomy, at which juncture an endolymphatic sac tumour was reported. Methods: A review of the world literature was carried out using Medline, which identified less than 150 reported cases of endolymphatic sac tumour. Conclusion: Endolymphatic sac tumours are rare lesions of the petrous temporal bone. Although benign, they can be locally destructive. At present, there is no consensus regarding the management and long-term follow up of these tumours. Surgical resection is usually favoured, although treatment with radiotherapy and gamma knife surgery has also been reported.