We report a 34-year-old male neuroBehcet's Disease (NBD) patient with atypical magnetic resonance imaging (MRI) findings, whose behavioral problems were followed by progressive neurological symptoms. The patient was hospitalized due to forgetfulness, irritability, behavioral dyscontrol and a choking sensation. T2-weighted MRI showed prominent atrophy of cerebellar hemispheres, the cerebellar peduncle, the midbrain and the pons. He was diagnosed with NBD after an evaluation of his medical history together with neuropsychiatric and laboratory findings. There are few reports of NBD with only brainstem and cerebellar atrophy. We discuss our patient in the context of the four previously reported cases. In NBD without evident mucocutaneo-ocular symptoms, neurologists should always consider the medical and family history. Early diagnosis of NBD helps to initiate appropriate treatment, thereby modulating the course of the disease and preventing complications. (C) 2008 Elsevier B.V. All rights reserved.