Background: Neurosarcoid affects approximately 5% of patients with sarcoidosis. A significantly more rare entity, necrotizing sarcoidosis affecting the central nervous system, has been confirmed previously in only three case reports. This paper documents three additional cases of necrotizing neurosarcoid, involving a wide spectrum of central nervous system (CNS) locations. Results: One patient presented to the emergency department after being found unresponsive. The second patient was referred due to hearing loss and the third patient sought care due to weakness and numbness of his left lower extremity. Locations of involvement were diverse and included diffuse leptomeningeal involvement, a cerebellopontine angle mass and a thoracic spinal cord lesion. All patients eventually underwent surgical biopsy, and histologic review of tissue samples revealed necrotizing granulomatous inflammation. Serum ACE levels were available for two of the patients and were within normal limits. Once the diagnosis of necrotizing neurosarcoid was confirmed, all patients were treated with systemic corticosteroid therapy; one patient was also treated with an immunosuppressive agent. Conclusions: Necrotizing neurosarcoid may occur more commonly than previously described and should be considered in the differential diagnosis of patients without systemic manifestations of sarcoidosis. ©2007 Dustri-Verlag Dr. K. Feistle.