本文报告一例罕见的、典型的神经-皮肤黑色素沉着病的临床病理资料,此患者出生时即有全身多处皮肤巨痣及黑痣斑。神经系统症状于生后31年出现;表现为脑膜黑色素细胞广泛浸润脑膜及脑室系统,引起脑脊液循环吸收障碍而发生交通性脑积水所致的颅压增高症状及脑膜炎症状。患者家庭多个成员有皮肤痣发生,从家系图分析,符合常染色体显性遗传方式。 This article reports a rare, typical clinical picture of neuro-cutaneous melanocytic disease, which occurs at birth with numerous giant skin nevi and mole nevus. Neurological symptoms appear after 31 years of life; the performance of meningococcal melanoma cells extensive infiltration of the brain and ventricular system, causing cerebrospinal fluid circulation disorders caused by traffic hydrocephalus caused by increased symptoms of intracranial pressure and meningitis symptoms. Multiple members of the patient family have skin nevi, from pedigree analysis, in line with autosomal dominant inheritance.