A case of hypertrophic cranial pachymeningitis was reported. A 58-year-old female presented the symptoms of headache and vomiting. At the age of 27, she had suffered from tuberculosis. Neurological examination on admission revealed bilateral papilledema, bilateral hearing disturbance, right hypoglossal nerve palsy, ataxic gait, and bilateral intentional tremor. CT scan showed dilatation of the lateral and third ventricles, and compression of the fourth ventricle with marked enhancement of cerebellar tentorium. A ventriculoperitoneal shunt was installed bringing about improvement in bilateral papilledema, ataxic gait, and bilateral intentional tremor. One month later, ataxic gait and bilateral intentional tremor recurred, and monoparesis of the left upper extremity developed. MRI demonstrated hypertrophic dura mater in the posterior fossa and compressed cervical spinal cord. Decompressive surgery was performed bringing about remarkable clinical improvement. The pathological specimen showed thickening of the dura mater with concentric layers of dense fibrous tissue infiltrated with plasma cells. A diagnosis of hypertrophic cranial pachymeningitis was established. Three years later, the clinical features were found unchanged, but contrast enhancement of cerebellar tentorium had progressed markedly. Hypertrophic pachymeningitis is a uncommon disease. But it should be noted that intracranial involvement is very rare. The etiology, symptomatology, neuroradiology, and treatment are discussed and the literature is reviewed.