We present a unique case of biopsy-proven necrotising sarcoidosis involving the central nervous system in a man in his 40s. The patient presented with a 2-week history of right-sided headache and diplopia. He had right trochlear and abducens nerve palsy, sensory blunting over V1 and V2 segment of right trigeminal sensory nerve and right sensory neural hearing loss. A contrast-enhanced MRI revealed an enhancing dural-based mass lesion in the petroclival area suggestive of probable meningioma. Surgical resection was attempted and intraoperative consultation with frozen section revealed granulomata. So, the lesion was biopsied and surgical intervention was terminated. A diagnosis of necrotising neurosarcoidosis was confirmed on histopathology. He was treated with steroids after excluding other causes of intracranial necrotising granulomas like tuberculosis and he clinically responded favourably. We report one of the very few case reports of histologically proven necrotising sarcoidosis involving the central nervous system mimicking petroclival meningioma.