Neurocutaneous melanosis(NCM)is a rare congenital syndrome characterized by congenital melanocytic nevus and melanocytic proliferations within the central nervous system(CNS),generally presents neurological symptoms within the first two years after birth.About half of NCM patients develop central nervous system melanoma.Imaging examinations have shown significant advantages in the diagnosis of NCM,especially magnetic resonance imaging(MRI),which has important value in early detection and diagnosis for NCM.This article provides a review of research status and progress in clinical and imaging diagnosis of NCM.