A 52-year-old woman presented with headaches, difficulty with word finding and left eye blindness. MRI showed enhancing frontal dural-based masses suggestive of meningiomatosis. Biopsy and debulking revealed necrotising granulomas, without discernible micro-organisms or neoplasia; a thorough clinical work-up was negative for infection and vasculitis. A CT scan showed mild bilateral hilar lymphadenopathy, a tiny pulmonary nodule and cirrhotic liver. Her subsequent alteration of mental status was attributed to hepatic encephalopathy based upon elevated aminotransferase and ammonia levels, biopsy evidence of hepatoportal sclerosis with rare granulomas and response to lactulose. A diagnosis of neurosarcoidosis of the necrotising sarcoid granulomatosis variant (NS-NSG) with atypical systemic involvement was made. This is the fifth case report of NS-NSG clinically mimicking a neoplasm and histologically mimicking a mycobacterial infection. NS-NSG can have an atypical clinical picture including intracranial masses; a thorough work-up to exclude infectious and other non-infectious aetiologies is a prerequisite to its diagnosis.