The term 'rhombencephalitis' refers to inflammatory diseases affecting the hindbrain. We experienced a case of rhombencephalitis in a 14 years old girl, who presented with fever, headache, and intractable hiccup for one week prior to admission. Brain MRI on admission showed bilateral high signal intensities in the dorsal portion of the medulla oblongata on T2WI and FLAIR. Examination of cerebrospinal fluid showed increased WBC count (33/mm3, Lymphocyte 81%), normal protein and glucose level. Immunoserologic study showed decreased C3, C4 level and increased Anti-ds DNA level. However, FANA was negative. The hiccup was controlled by administration of IVIG (1 g/kg/day for 2 days) and steroid (30 mg/kg/day for 3days) without any neurologic symptoms. We report a case with the brief review of related literatures.