We report a case of giant single-hole direct arteriovenous fistula (AVF) located in the posterior fossa of a child with Rendu-Osler-Weber disease (ROW). There is neither a clinical nor an angioarchitectural difference between AVFs associated with ROW and sporadic AVFs, but ROW should be suspected in children with multifocal pial AVFs. Transarterial embolization was performed to obliterate the AVF in a unique procedure using simultaneous bifemoral catheterization. At follow up 4 years later, the clinical examination was normal. Control MR scans and angiography showed the total occlusion of the fistula, permanent thrombosis of the venous pouch, and disappearance of the abnormal venous drainage. In this case, the endovascular procedure was available and we estimated that it represented a lesser risk than the surgical approach (risk of hemorrhage and risk of thrombosis involving the feeding arteries and the brain stem venous drainage). Furthermore, it avoids craniotomy and reduces the duration of hospitalization.