Osler-Weber-Rendu syndrome (OWR) is an autosomal dominant disorder characterized by recurrent epistaxis, mucocutaneous or visceral telangiectasias, and arteriovenous malformations in the lungs, liver, brain, and gastrointestinal tract. Hepatic artery aneurysms (HAAs) can also occur in OWR patients. HAAs are the second most common type of visceral artery aneurysm, and mortality rates are high owing to the lack of a tamponade effect. Anatomical variations of the celiacomesenteric vasculature are common, and the most common variation is that of the right hepatic artery originating from the superior mesenteric artery (SMA). We present the endovascular treatment of a patient with OWR and an aberrant right HAA originating from the SMA, with coil embolization and stent grafting. Giant HAAs can be treated endovascularly. However, stent graft placement should be reconsidered because of the need for antithrombotic medication, which may increase the incidence of epistaxis attacks in that patient group.