Acute disseminated encephalomyelitis (ADEM) is an inflammatory and demyelinating condition of central nervous system. A wide range of neurological symptoms are present along with multifocal lesions within brain and spinal cord. Clinically, severe focal or multifocal neurological deficits with an abrupt onset may develop. The most severe form of Behçet\'s disease is the one with neurological involvement, which constitutes 20% of all Behçet\'s cases. Rarely, central nervous system involvement may develop before any other organ systems. In this work, we aim to report a Neuro- Behçet case presented with ADEM who did not have any previous diagnosis.