A 62-year-old woman presented with raised intracranial pressure and features of a right cerebellopontine angle tumour with extension into the right middle cranial fossa. The patient died before a surgical excision could be performed. The autopsy revealed a primary esthesioneuroblastoma of the sphenoid sinus eroding the petrous bone and extending into the middle cranial fossa with metastatic tumour in the liver, and paratracheal and hilar lymph nodes. Although rare, esthesioneuroblastoma must be considered in the differential diagnosis of petrous-sphenoid lesions.