A 76-year-old man with herpetic vesicle in the right auricle developed ipsilateral 5th, 6th, 7th, and 8th cranial nerves involvement and pain in the dermatome of the second cervical nerve. The CSF study revealed elevated opening pressure up to 220 mmH2O, and pleocytosis up to 151 cells/mm3. Ninety-nine percent of the CSF cells were mononuclear cells. CSF protein was 47 mg/dl, and CSF glucose was 62 mg/dl. On the 24th hospital day the CSF cells decreased to 13/mm3 with 100% mononuclear cells. Titer of varicella-zoster virus (VZV) antibody was significantly elevated in CSF. Brain MRI and ABR demonstrated no abnormality. Although disorders of 5th and 6th cranial nerves and second cervical nerve improved, mild facial nerve palsy lasted and hearing disturbance showed no recovery. There are only seven cases of Ramsay Hunt syndrome associated with external ophthalmoplegia in the literature. However, un like the present case, none of these cases presented disorders of upper cervical nerves. In this case, we speculate that spreading of reactivated VZV caused local meningitis and multiple cranial nerve involvement as well as the second cervical nerve.