A 20-year-old man noticed enuresis since the age of 18 years. He was also pointed out gait unsteadiness by his friends. Neurologic examination revealed spastic tetraparesis with mild cerebellar ataxia. The cerebrospinal fluid had mild pleocytosis containing neutrophils and brain magnetic resonance imaging showed mild pontocerebellar atrophy. Urodynamic study revealed uninhibited bladder and detrusor-sphincter dyssynergia. The diagnosis of neuro-Behçet's disease was made by the past history of recurrent genital and aphthous ulcers and positive HLA-B51. The frequency of enuresis was reduced by the oral administration of corticosteroid. We speculated the patient's enuresis was caused by the central nervous disinhibition at night together with uninhibited bladder.