Pulmonary lesions, with sarcoid-like granulomas exhibiting non-caseous
necrosis, with associated granulomatous arteritis fulfill the diagnos
tic criteria of necrotizing sarcoid granulomatosis (NSG). We report th
e case of a woman who presented with recurrent headaches, transient ri
ght hemipareses and left-sided ophthalmoplegia. An excised left retro-
orbital lesion demonstrated sarcoid like changes, and the illness resp
onded to steroid therapy. Twelve years later, the patient developed a
tumour in the right lung. The resected specimen showed the histologica
l hallmarks of NSG, and careful review of the retro-orbital lesion, re
moved 12 years previously, revealed similar histology. Extrapulmonary
involvement in NSG is rare and has been histologically proven on only
one previous occasion. The presentation of necrotizing sarcoid granulo
matosis in two different systems 12 years apart is unusual and was co
nsidered worth reporting.