We report the case of a child's Jadassohn sebaceous noevus, localised on the vertex cranii, the trunk and the limb, associated with the presence of ophthalmological and neurological abnormalities. The diagnosis of this syndrome has been difficult because all the signs were not present at birth. The surgical treatment of the vertex cranii lesions will be realised after expansion with sub cutaneous prothesis, allowing a large exeresis and an adequate reconstruction. Only the anatomo-pathological examination allows any malignant degeneration of the lesion.