A rare tumour in the cerebellopontine angle: endolymphatic sac tumour

We present a case of a papillary tumour at the cerebellopontine angle in a 54-year-old man. He presented with right-sided ear pain associated with dizziness and hearing loss. The radiological diagnosis was in favor of acoustic neurinoma. Surgical excision was performed and the diagnosis of the endolymphatic sac tumour was made. Endolymphatic tumour is a low grade adenocarcinoma that originates from the endolymphatic sac. The definitive diagnosis requires a combination of clinical features, radiological finding and pathological correlation.