A case of hypertrophic cranial pachymeningitis associated with intramedullary lesion was reported. A 57-year-old male presented with the symptoms of Jacksonian seizure and weakness of right lower extremity. Neurological examination showed weakness and muscular atrophy of right lower extremity (MMT 1-2) and hyperreflexia. Superficial sensation and position sense were normal, however cortical sensory disturbance was recognized in his right lower extremity. MRI revealed diffuse dural thickening with gadolinium enhancement in the left convexity and hyperintense lesion in the bilateral (left dominant) frontal to parietal lobe on T2 weighted image. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) showed occlusion of the superior sagittal sinus. Histological examination revealed extensive fibrous tissue with lymphocytes infiltration around the vessels. The cause of the intramedullary lesion in this patient may have been related to the occlusion of superior sagittal sinus, due to thickening dura mater and influence of inflammation.