Introduction:

Vein of Galen aneurysm is a rare and congenital cerebral arteriovenous abnormality with reported incidence of 1:25 000 live births. It represents the 30 % of the vascular congenital cerebral malformations that harm the pediatric population.

Objective:

To show updated information about the topic.

Method:

The information was obtained from the automated search mostly done in MEDLINE, CurrentContents and Scielo databases.

Development:

Vein of Galen malformation presents with symptoms and signs derived from the severe refractary heart failure to medical treatment and the neurologic damage it causes. That entity implies severe morbidity and mortality on the neonatal period. Angioresonance is the reference technique, but the Doppler ecography is a good valuation method. The election treatment is the endovascular embolization.

Final considerations:

There must be an elevated rate of suspect to do not fail on the early diagnostic of the disease. It is important to define the lesion’s anatomy, because of the clinic, therapeutic and prognostic implications this represent. The successful treatment is still a complex therapeutic challenge.

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