Endolymphatic sac tumour occurring in a 32-year-old man presenting with Meniere's like symptoms of recurrent vertigo, hearing loss and tinnitus is described. Magnetic resonance imaging and computed tomography showed a vascular bone tumour centred over the retrolabyrinthine aspect of the temporal bone where the endolymphatic sac was located. Surgical excision via a translabyrinthine approach was performed. Endolymphatic sac tumours are rare papillary adenocarcinomas that arise from the endolympatic sac. It can be mistaken both on radiology and histology for other tumours such as paragangliomas, renal or papillary thyroid carcinoma metastases. Surgical excision is the treatment of choice but sacrifice of the auditory and facial nerve may be needed in advanced cases to achieve tumour clearance.