Background: Neurosarcoidosis is rarely preceded by impaired oculomotor symptoms. Purpose: To report a case of neurosarcoidosis that showed diplopia as the initial manifestation and that responded to systemic gamma globulin therapy. Case: A 73-year-old female presented with diplopia and weakness in her left leg. Corrected visual acuity was 1.2 in either eye. No abnormality was present in the anterior chamber, optical media or the fundus. The right eye showed abducens palsy and oculomotor paresis. Magnetic resonance imaging showed normal findings in the orbit and brain. She was diagnosed with chronic inflammatory demyelinating polyneuropathy and was treated by intravenous human immunoglobulin. Diplopia started to improve 9 days later. Computed tomography of the lung showed nodules that proved to be non-necrotizing granulomatous lymphadenitis leading to the diagnosis of sarcoidosis. Conclusion: Systemic immunoglobulin was effective for neurosarcoidosis with oculomotor disorder.