Neurocutaneous melanosis is considered to follow from neurulation disorders which could account for associated developmental malformations as the so-called Dandy-Walker complex. Cutaneous lesions are usually recognized at birth whereas neurological manifestations develop later. Numerous neurological symptoms have been reported according to extent of leptomeningeal and parenchymal infiltration. Whether magnetic resonance imaging of the neuroaxis represents the choice radiological exam, definite diagnosis relies upon the histological data obtained by mean of biopsy. Once symptomatic, surgical and medical measures remain palliative since death occurs within 3 years.

We believe that the timing of treatment in the course of the disease and selection between radiosurgical versus microsurgical procedure are key issues in the management of IVS. Preservation of hearing and good facial nerve function in surgery for VS is a reasonable goal for many patients with intracanalicular tumors and serviceable hearing. Once open surgery has been decided, selection of the approach mainly depends on individual anatomical considerations and experience of the surgeon.

Aberrant monozygotic twining leading to inclusion of a degenerate fetus inside its twin bearer results in FIF or teratomas. Surgical removal represents the main treatment. Intracranial FIF remains an extremely rare congenital condition, with a favorable oncological prognosis; the developmental prognosis appears conditioned by the absence of other malformation, the small size of the lesion, and the early management of intracranial hypertension.