We report a case of acute optic neuropathy with Behçet's disease (BD) in a 27-year-old man. The patient experienced two episodes of acute optic neuropathy in one year. The first was in the right eye and the second was in the left eye. In both episodes, several months before acute optic neuropathy developed, oral and genital ulcers, erythema nodosa and epididymitis were noted. The patient was diagnosed with BD. High-dose systemic corticosteroid resulted in clinical improvement in both episodes. Neurological involvement in BD is well known. Brainstem disturbance, meningomyelitis and palsy involving cranial nerves VI, VII have been reported. However, optic neuropathy in BD is very rare. This case suggests that optic neuropathy can affect both eyes and be recurrent. This case provides clinical insight into optic nerve involvement in BD.