Behçet’s disease is a multisystem vasculitis. Its neurological involvement mostly includes parenchymal and non-parenchymal central nervous system manifestations. Peripheral nervous system presentations are rare. A 32-yr-old male patient who fulfilled the international study group criteria for Behçet’s disease, referred to our center with walking difficulty and repeated falling downs. Neurological examination revealed weakness, wasting and fasciculation of distal muscles associated with corticospinal signs. Neuroimaging data were insignificant but electrodiagnostic studies were in favor of motor neuron disease. To best of our knowledge, this is the first report of association of amyotrophic lateral sclerosis (ALS) and Behçet’s disease.