Plastic surgeons who treat congenital giant nevi should be aware of the neurocutaneous melanosis syndrome and its variants. When neurologic symptoms are present concurrent with MRI evidence of central nervous system involvement, treatment of the cutaneous lesion must be tempered by knowledge of a poor prognosis. In the otherwise asymptomatic patient, the plastic surgeon should consider screening with MRI imaging, realizing that the clinical significance of a positive MRI scan without neurologic symptoms is unknown.