We explore the clinical characteristics of neurocutaneous melanosis (NCM) in adult patients to further understanding of the disease. Methods We present a rare case of an adult patient suffering from neurocutaneous melanosis with anepia and review the Chinese and English literature to analyze the clinical features of adult neurocutaneous melanosis patients in China. Results: From Jan 1995 to April 2022, there were thirty-four adult NCM patients, aged 19 to 59 years, average 26.9 years old, twenty-three males (67.6%), eleven females (32.4%), two cases of diffuse melanocytosis (5.8%), thirty-one cases of malignant melanoma (91.1%)) and one case of mixed nevus (3.1%). Twenty-nine cases showed satellite nevi (73.5%), and five cases were unknown (26.5%). Thirty-three cases had obvious intracranial lesions(97.0%), and one case had no intracranial lesions (3.0%). Nine cases of intracranial hypertension (26.4%), ten cases of epilepsy(29.4%), one case of hydrocephalus (2.9%), one case of cerebral hemorrhage (2.9%), one case combine Dandy-Walker deformity (2.9%). Conclusion: Neurocutaneous melanosis (NCM) is a very rare disease, especially in adults. Neurosurgical intervention can help improve diagnosis of this disease. Further evidence is needed to establish the negative associations of NCM incidence with oestrogen levels.