Neurosarcoidosis, rare in patients with sarcoidosis, may present with protean manifestations according to the regions of involvement from peripheral nerves to the central nervous system. Cavernous sinus is rarely involved by sarcoidosis, and it can result in different cavernous sinus syndromes based mainly on the involvement of the trigeminal nerve. We report a 54-year-old man with pulmonary sarcoidosis and cavernous sinus syndrome and review the clinical course, laboratory findings, and neuroradiologic features of the condition. This patient presented with complete ophthalmoplegia of left eye. Magnetic resonance imaging revealed a lesion with gadolinium-enhancement in the left cavernous sinus. Serial chest examinations showed bilateral hilar enlargement. Pulmonary sarcoidosis was diagnosed according to the findings of lymph nodes biopsies. Elevated erythrocyte sedimentation rate and serum angiotension converting enzyme level were observed. After steroid administration, his ocular palsy ameliorated in a few days and cavernous sinus lesion completely disappeared within 3 months after treatment. Although rare, neurosarcoidosis should be considered in the differential diagnosis of cavernous sinus syndromes with neuro-ophthalmologic signs. For early diagnosis of neurosarcoidosis, it requires a high index of suspicion for searching sarcoidosis at sites outside the nervous system. Corticosteroid treatment is generally followed by improvement in clinical status, but there is a high rate of progression and recurrence after the treatment.