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Introduction: A vein of Galen aneurysm (VGA) is a rare, congenital, intracranial vascular malformation often diagnosed in the perinatal period. In recent years there have been international reportings of good results after endovascular embolization during the first year of life. Materials and methods: In this paper the clinical course of 10 children born in Denmark with perinatal presentation of VGA is reviewed. Results: 2 out of 4 children who underwent endovascular embolization during the first year of life survived. 1 became moderately motorically and mentally retarded - the other severely. No attempt was made to close the VGA in 6 children, either because it was impossible to stabilize them, or because other kinds of severe intracerebral damage were already present. Conclusion: 9 out of the 10 children with a perinatal presentation of VGA are either deceased or have severe motor function and mental damage. Despite international reportings it is still recommended to carefully consider whether or not it is ethically correct to continue treatment if a neonate with VGA cannot be haemodynamically stabilized within the 1st week of life despite intensive care, or if massive intracerebral damage is already present. If a neonate is stabilized, specialized units that perform endovascular embolizations for VGA during the 1st year of life must be contacted for an assessment in preparation for treatment.

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