We present a patient with Bickerstaff's brainstem encephalitis in whom MR imaging correlated with the clinical findings during the course of the disease. T2-weighted MR imaging showed increased signal intensity in the medulla oblongata, upper pons, pendunculi cerebelli and the cerebellum. In the course of the disease the lesions on MRI tended to descend more caudally. No anti-GQ1b antibody titers could be detected in the acute phase of the illness. Our findings suggest that MRI is the most valuable diagnostic tool in supporting the clinical diagnosis of Bickerstaff's brainstem encephalitis and may be helpful in the distinction between Miller Fisher syndrome and Bickerstaff's brainstem encephalitis.