BACKGROUND Less than 1 per cent of tumours occurring in the region encompassing the internal auditory canal and the cerebellopontine angle are malignant. Primary central nervous system melanomas arising from this region are exceptionally rare and are often initially misdiagnosed as acoustic neuromas. METHODS We present a 71-year-old man with acute vestibular disturbance and unilateral hearing loss. Magnetic resonance imaging demonstrated a mass, thought to be a cochlear nerve schwannoma, involving the cochlea and the internal auditory canal. At surgery, a pigmented mass adherent to the facial nerve was visualised, and the observed histopathology was consistent with a malignant melanoma. No extracranial site for the primary tumour was found, suggestive of a primary central nervous system melanoma. RESULTS Despite surgical resection and adjuvant radiotherapy, the patient re-presented with extensive leptomeningeal disease 16 months later. CONCLUSION Malignant tumours in the internal auditory canal and cerebellopontine angle region are rare. Early diagnosis and management are aided by recognition of characteristic factors such as a history of prior malignancy, atypical magnetic resonance imaging findings and accelerated audiovestibular symptoms. Despite the presented patient's outcome, total surgical resection with post-operative radiotherapy remains the recommended treatment.