A Case Report of Neurocutaneous Melanosis with Associated Dandy-Walker Complex

 Neurocutaneous melanosis (NCM) and Dandy-Walker malformation (DWM) are two forms of rare congenital neurodysplasia. NCM is a rare dysmorphogenesis characterized by single or multiple giants pigmented cutaneous nevi and the involvement of benign and/or malignant melanocytic tumors of the leptomeninges. DWM is a rare congenital malformation of the brain. Cystic enlargement of the fourth ventricle is its characteristic that communicates with an enlarged posterior fossa, cerebellar dysgenesis, high tentorial insertion, and hydrocephalus. However, these two conditions are rare, and NCM associated with DWC is even more unusual.

Case Report: 

Here, we report a male newborn with macrocephaly and multiple pigmented nevi over his whole body with regular borders and normal weight, height, and spine. He was finally diagnosed as NCM in association with DWC.

 After diagnosing NCM in association with DWC, appropriate follow-up is recommended; however, there is no particular treatment to prevent the malignant change.