Introduction. Headache opposite to a blue nevus of the scalp can revea
l intra cranial melanotic lesions. Case report. A 25-year-old man cauc
asian was admitted to hospital for a first generalized tonic-clonic se
izure. For six months, he has had episodic frontal-temporal right head
ache opposite to a blue pigmentary cutaneous congenital lesion in fron
tal territory (histology confirmed benign blue nevus). Neurologic exam
ination noted a right congenital hereditary ophtalmoplegia. Cerebral M
RI showed a right rolandic tumor with diffuse leptomeninge infiltratio
n. This patient was operated of a meningeal melanocytoma with leptomen
inges melanosis. Discussion. The apparition of headache related to a b
lue nevus must lead to realize a cerebral MRI to look for a neuroectod
ermic hamartoma: melanotic tumor (in particular melanoma), or leptomen
inges melanosis with high potential of degeneration. Meningeal melanoc
ytoma is a rare benign spinal or intra cranial melanotic tumor.