To analyze the clinical and imaging features of hypertrophic cranial pachymeningitis (HCP) . Methods: One case of HCP was reported, and the relevant literature was reviewed and retrospectively analyzed. Results: The patient experienced headache at the onset of illness along with decreased visual acuity. Hematology test: ANA (+ ). Lumbar puncture: cerebrospinal fluid pressure ≥330 mmH2O. Cerebral MRI, plain and enhanced: cerebral falx and tentorium of cerebellum, bilateral frontal parietal dura thickening and enhancement. Dural biopsy: fibrous hyaline tissue scattered around lymphocytes and plasma cells, most apparent surrounding blood vessels. Immunohistochemistry: CD138 (+ ), IgG (individual cells + ), IgG4 (individual cells + ). Hormone therapy significantly improved symptoms. We collected data of 189 cases of HCP reported in the last 5 years. Headache is the most presented initial symptom. Dural hypertrophy and enhancement in different parts of the head was observed in all patients by plain and enhanced cranial MRI. After hormone treatment, combined hormone and immunosuppressive treatment, or surgical treatment, more than 90% of the patients' symptoms were improved. Conclusion: The clinical symptoms of HCP can be characterized by chronic headache, cranial nerve involvement, and ataxia. It is imperative to perform hematological examination, plain and enhanced cranial magnetic resonance imaging, and, when necessary, dura mater biopsy to make a diagnosis.