Abstract: 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.