Rhomboencephalitis is a potentially life-threatening condition due to inflammation of the hindbrain with an unpredictable outcome depending on the wide spectrum of etiologies and the promptness of diagnosis and treatment. A 23-month-old Caucasian male presented with fever, clouding of consciousness, and trunk ataxia. Three weeks earlier he received active immunization against varicella-zoster virus. Routine laboratory tests were unremarkable. Cerebrospinal fluid showed might elevation of leukocytes. The infection panel was negative. Brain magnetic resonance showed signal hyperintensity in the dorsal portion of the pons, which was consistent with a rhomboencephalitis. Autoimmune pathogenesis was supposed and a high dose of steroids was started. The patient's neurologic status progressively improved till full recovery and complete regression of previous magnetic resonance lesions after 1 year. Nevertheless, longer follow-up is needed in order not to miss any possible signs of an underlying autoimmune neurologic disorder.