We describe imaging findings in a 2-year-old girl,vith neurocutaneous melan
osis and malignant cerebral melanoma. Because the cerebral melanoma in this
child was of the amelanotic type, high-signal intensity on unenhanced T1-w
eighted images was not present. The cutaneous lesions played a crucial role
in establishing a correct (presumed) histopathologic diagnosis on the basi
s of the imaging findings. To our knowledge this is the first report descri
bing an intracranial amelanotic malignant melanoma in association with neur
ocutaneous melanosis.