Sudden change of a large congenital melanocytic nevus to neurocutaneous melanosis

Neurocutaneous melanosis (NCM) is a congenital phakomatosis in which large congenital melanocytic nevi are associated with a benign or malignant melanocytic tumor of the leptomeninges. Because the prognosis of patients with symptomatic NCM is poor, it is essential to monitor the large congenital melanocytic patient regularly for a neurological evaluation. However, it has not been reported how quickly the lesion could appear. We observed a case of NCM suddenly developing in a large congenital melanocytic nevi patient. With this case, the NCM had developed within six months and was aggravated during the subsequent six months.