A 43-year-old male with a history of eosinophilic angiocentric fibrosis presented with headache and double vision since 48 h. Idiopathic hypertrophic pachymeningitis was diagnosed based on nuclear magnetic resonance and comprehensive systemic study findings. Eosinophilic angiocentric fibrosis and idiopathic hypertrophic pachymeningitis have been associated with IgG4-related diseases, but this was the first case in which they coexisted in the same patient. After steroid treatment failure, rituximab was used with excellent results.
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