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Malignant mesothelioma is an uncommon neoplasia which primarily involves the pleura or peritoneum. Central nervous system involvement is rare. A rare presentation of metastatic pleural mesothelioma, which had infiltrated the meninges and brainstem, is described. The patient presented with diplopia following a 2-week history of malaise, myalgia, mild headache and diarrhoea. Clinical examination found global areflexia, cerebellar ataxia and bilateral sixth nerve palsies. Differential diagnoses included the Miller–Fisher variant of Guillain–Barre syndrome, malignant meningitis and infectious meningitis. The patient was treated with immunoglobulins, plasmaphoresis and corticosteroids; however, he deteriorated and died 31 days after admission. Retrospective examination of the MRI of the brain found diffuse low attenuation changes within the pons and cerebral peduncles. Postmortem examination favoured a diagnosis of an early sarcomatoid malignant mesothelioma of pleural origin with leptomeningeal metastatic deposits.