We report the treatment and follow-up, including magnetic resonance imaging (MRI) of a patient with idiopathic hypertrophic pachymeningitis and review the literature published in 21th century, with emphasis on the management and clinical outcomes of this rare disorder. Hypertrophic pachymeningitis is extremely rare. It is a fibrosing inflammatory process which involves the dura mater. Numerous pathological entities produce thickening of the pachymeninges. Thus, idiopathic hypertrophic pachymeningitis is diagnosed by exclusion. We present a case of patient with idiopathic hypertrophic pachymeningitis who had varied clinical presentation. Imaging studies revealed diffuse thickening of the pachymeninges.