目的　提高对儿童原发脑脊膜黑色素瘤病的认识。方法　回顾分析1例原发脑脊髓膜黑色素瘤病患儿的临 床资料，并复习相关文献。结果　女性患儿，11岁，临床表现为头痛、呕吐、抽搐发作。头颅磁共振成像示弥散性脑膜增厚， 脊髓磁共振成像示多发小结节；脑脊液细胞学检查可见黑色素细胞。确诊为原发脑脊膜黑色素瘤病。结论　原发脑脊膜 黑色素瘤病罕见，当临床出现不能解释的颅内压增高时，需警惕原发脑脊膜黑色素瘤病可能。

Objective　To improve the understanding of primary leptomeningeal melanomatosis (PLM). Methods　The clinical data of a child with PLM were retrospectively analyzed, and pertinent literatures were reviewed. Results　An 11 years old female child, whose main clinical manifestations were headache, vomiting and seizures. Brain MRI showed diffuse meningeal thickening, and spinal cord MRI showed multiple nodules. Cerebrospinal fluid cytology detected the melanocytes. The patient was diagnosed with PLM at 5 weeks. Conclusions　PLM is rarely seen, the diagnosis may be challenging even for experiences neurologists. When the appearance of unexplained intracranial pressure increases, it is important to be aware of the possibility of PLM.