The article presents a case of idiopathic hypertrophic pachymeningitis of a 61-year-old male patient with severe cephalgia and progressive neuropathy of the oculomotor nerves. The diagnosis was confirmed by MRI with gadolinium, which revealed thickening of the dura mater with accumulation of paramagnetic in the convexital parts of the frontal and temporal regions, as well as on the base of the skull and tentorium. During the use of pulse therapy with glucocorticosteroids (GCS) the symptoms regressed, but when the therapy was stopped, there was a relapse of ptosis and oculomotor abnormalities on the other side followed by a slower effect of GCS therapy. The article also presents a brief review of current knowledge about this pathology.