Neuro-Beh double dagger et's disease (NBD) is a rare clinical entity. There are no reports about cortical atrophy in NBD. We report a patient with NBD exhibiting only cortical and cerebellar atrophy without any lesions. A 38-year-old male was hospitalized due to gait disorder. He had experienced forgetfulness, irritability, sexual and behavioral dyscontrol, and incontinence for 5 years. Magnetic resonance imaging (MRI) showed cortical and cerebellar atrophy, but there were no lesions. In the differential diagnosis of NBD, physicians should take into account the presence of both brainstem and cerebellar and cortical atrophy even in the absence of typical lesions for NBD, particularly in the presence of cognitive dysfunctions.