RESUMEN
De novo arterio-venous malformations (AVMs) of the brain have been rarely previously reported, especially in the pediatric population. Although AVMs have possible connections with other diseases, the association with congenital portosystemic shunt (CPSS) has never been reported before. A child was followed for CPSS and cutaneous and hepatic angiomas. Brain MRI and angiography revealed an AVM within the left temporal region that was not present at a previous MRI. The patient underwent successful resection of the AVM. This case adds new evidence on the complex variety of diseases associated with multisystemic vascular malformations corroborating the hypothesis of a multifactorial origin of de novo cerebral AVMs, under a possible common genetic substrate.