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Background.. Idiopathic hypertrophic cranial pachymeningitis is a rare inflammatory disease of the dura mater of unknown origin with frequent recurrences, progressive cranial nevre palsies and headache despite initial response to steroid therapy and may cause sequels.
Observation.- We report a two-year followup of a patient having recurrent multiple cranial nerve palsies whose radiological and clinical features were consisted with pachymeningitis. Her initial symptomatic response to steroids disappeared during the course of the disease. In accordance with the patient who had the diagnosis of idiopathic hypertrophic pachymeningitis (IHP) due to absence of any etiological causes, we discussed the diseases to be considered in the differential diagnosis of IHP.