© 2020 Elsevier Inc. Encephalitis is a relatively challenging rare condition caused by a diverse group of etiologies. Brainstem encephalitis/Rhombencephalitis (BE), which affects the cerebellum, pons, and medulla, is even less common and more challenging for diagnosis and treatment. At this time, there is scattered data about BE in the literature, mainly in the form of case reports and case series. In this manuscript, the imaging presentation of BE is reviewed with the help of case examples. Many imaging presentations are not pathognomonic for BE; however, in many cases, clinical presentation, the spatial distribution of lesions, and other associated radiological lesions can provide the radiologists and clinician the clues to an accurate diagnosis.