Publication date: Available online 13 April 2017 Source:Seminars in Pediatric Neurology Author(s): Brittany Brand, Dave Somers, Blake Wittenberg, Jessica Gautreaux, Stephen Deputy Idiopathic hypertrophic pachymeningitis is a rare disorder of diffuse thickening of the cranial or spinal dura matter without an identifiable cause. Most common in adult males, idiopathic hypertrophic pachymeningitis typically presents with headache with or without varied associated focal neurologic deficits and findings of dural enhancement on MRI in a linear, nodular or combined pattern. As it is felt to be an autoimmune disorder, treatment with high dose corticosteroids is typically recommended, and without intervention, the course is usually progressive. The disease can commonly progress with a relapsing remitting course requiring other immune modulators such as methotrexate, azathioprine, or cyclophosphamide for control. Here, we describe a unique case of idiopathic hypertrophic pachymeningitis as it presented in a pediatric patient and resolved without immunmodulatory therapy.