A patient with Ehlers-Danlos syndrome type IV had a celiac artery aneurysm. There are only nine previously published cases of visceral artery aneurysms in this condition, and surgical management was used in only four of them. This is, we believe, the first published report of a successfully treated celiac artery aneurysm in this condition in which the hazards of treatment and our rationale are explained. The literature of this rare problem is also reviewed.