The term rhombencephalitis refers to inflammatory diseases affecting the 
hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of 
etiologies, including infections, autoimmune diseases, and paraneoplastic 
syndromes. Infection with bacteria of the genus Listeria is the most common 
cause of rhombencephalitis. Primary rhombencephalitis caused by infection with 
Listeria spp. occurs in healthy young adults. It usually has a biphasic time 
course with a flu-like syndrome, followed by brainstem dysfunction; 75% of 
patients have cerebrospinal fluid pleocytosis, and nearly 100% have an abnormal 
brain magnetic resonance imaging scan. However, other possible causes of 
rhombencephalitis must be borne in mind. In addition to the clinical aspects, 
the patterns seen in magnetic resonance imaging can be helpful in defining the 
possible cause. Some of the reported causes of rhombencephalitis are potentially 
severe and life threatening; therefore, an accurate initial diagnostic approach 
is important to establishing a proper early treatment regimen. This pictorial 
essay reviews the various causes of rhombencephalitis and the corresponding 
magnetic resonance imaging findings, by describing illustrative confirmed cases.