Behcet disease is a multisystemic, chronic, recurrent inflammatory disorder characterized as a triad of hypopyon uveitis, oral aphthae, and genital ulcers. Neurologic involvement in Behcet disease (neuro-Behcet) is common. Neuro-Behcet disease typically manifests late after disease onset, rarely coincides with ocular involvement, and often heralds a poor prognosis for final vision and survival. We present a case of neuro-Behcet disease with neurologic onset concomitant with systemic and ocular involvement that was responsive to treatment with interferon-alpha 2a.