Introduction: Headache opposite to a blue nevus of the scalp can reveal intra cranial melanotic lesions.
Case report: A 25-year-old man caucasian was admitted to hospital for a first generalized tonic-clonic seizure. For six months, he has had episodic frontal-temporal right headache opposite to a blue pigmentary cutaneous congenital lesion in frontal territory (histology confirmed benign blue nevus). Neurologic examination noted a right congenital hereditary ophtalmoplegia. Cerebral MRI showed a right rolandic tumor with diffuse leptomeninge infiltration. This patient was operated of a meningeal melanocytoma with leptomeninges melanosis.
Discussion: The apparition of headache related to a blue nevus must lead to realize a cerebral MRI to look for a neuroectodermic hamartoma: melanotic tumor (in particular melanoma), or leptomeninges melanosis with high potential of degeneration. Meningeal melanocytoma is a rare benign spinal or intra cranial melanotic tumor.