Endolymphatic sac tumours (ELST) are rare tumours of the petrous temporal bone. They may arise sporadically or be associated with von Hippel-Lindau disease. Their differential diagnosis is discussed. We present the clinical and histopathological features of two new patients with ELST and outline the management of their condition. In addition, we review a third case previously reported as a choroid plexus papilloma in which the histology has been re-assessed and the diagnosis changed to ELST. The controversy regarding the cellular origins of adenomatous tumours of the temporal bone is highlighted.