ABSTRACT
Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the development of congenital melanocytic nevi and benign or malignant melanocytic tumors of the central nervous system. Neurologic symptoms usually manifest during the first two years of life with a high mortality rate.This is a case of a 3-year-old girl presenting with generalized large and multiple hyperpigmented macules and patches, some with hair, over the scalp, face, trunk, and extremities, present since birth. Histologic findings of a nodule within a nevus include nested melanocytes at the epidermis, cords and strands of melanocytes extending to the deep reticular dermis showing maturation with descent and lack of mitosis consistent with melanocytic nevi. The cranial magnetic resonance imaging contrast study revealed melanocytic deposits on the cerebellum and amygdala. She has regular follow-up with her dermatologist, pediatrician, and neurologist. The patient has remained asymptomatic of neurological symptoms despite the melanocytic deposits in the central nervous system.