Vascular Ehlers-Danlos syndrome (vEDS) causes fatal vascular complications due to vascular fragility. However, invasive therapeutic procedures are generally avoided except in emergencies. We report a case of vEDS presenting with rapid expansion of a hepatic arterial aneurysm successfully treated using prophylactic endovascular therapy. A 43-year-old woman with vEDS confirmed by genetic testing was hospitalized for a symptomatic hepatic arterial aneurysm that expanded rapidly within a week. Prophylactic coil embolization was then successfully performed. Although the general applicability of this approach cannot be determined, prophylactic endovascular therapy can clearly be an option for arterial aneurysms at high risk of rupture.