The authors performed a systematic review of literature and describe the clinical signs and symptoms of endolymphatic sac tumours (ELST) in this article. A total of 113 patients and 118 cases from 26 studies were included in the study. The most common clinical presentation was hearing loss followed by vertigo, tinnitus, and facial nerve palsy. ELSTs are low-grade tumours originating from the epithelium of the endolymphatic duct and sac. They can occur sporadically or as part of von Hippel Lindau disease (VHL). Tumours associated with VHL have a female preponderance, are seen in younger patients and are more aggressive. Radiology and pathology results suggest that ELSTs arise from the proximal part of the endolymphatic sac. Both high resolution CT and magnetic resonance imaging help to diagnose and help in the surgical planning of the tumour. Treatment includes observation for slow growing tumours versus surgery for aggressive tumours. The authors conclude that fluctuating hearing loss, tinnitus and vertigo can confound and delay early diagnosis of ELSTs.