A 65 year old male, presented with ophthalmoplegia and reduced vision in his left eye. The magnetic resonance imaging (MRI) of the brain revealed three hyperintensity areas located in the left parasellar area, left lacrimal gland and right frontal bone. Chest CT revealed bilateral pulmonary masses. The pathological diagnosis was necrotizing granuloma and vasculitis. Nine months after the diagnosis, the right eye was involved. This case report presents a rare manifestation of blinding necrotizing sarcoid granulomatosis that had responded to steroid therapy, but had a relapsing clinical course.