Neurocutaneous melanosis (NCM) is associated most commonly with giant conge
nital melanocytic nevi (CMN), in particular those on the scalp or In a post
erior axial location that are accompanied by satellite congenital nevi. It
also can occur in patients with multiple medium-sized CMN. In general, the
prognosis of those with symptomatic NCM is poor, even in the absence of mal
ignancy, while the prognosis of those with asymptomatic NCM detected via sc
reening varies and is more difficult to predict. Herein we report an asympt
omatic patient with a giant CMN and multiple satellite nevi who had a scree
ning magnetic resonance Imaging (MRI) study at age 5 months that showed a r
ounded area of increased signal In the right temporal lobe on T1-weighted i
mages, suggestive of parenchymal melanosis. This melanotic mass was resecte
d at age 10 months, and histologic examination of the surgical specimen sho
wed prominent perivascular collections of benign, pigment-containing melano
cytes within cerebral tissue. The patient remains healthy 8 years later. Hi
s excellent long-term outcome and other reports of NCM with localized centr
al nervous system (CNS) involvement apparent on MRI may have implications f
or management, including early imaging of patients with high-risk CMN and p
otential surgical intervention for NCM.