Congenital internal carotid artery (ICA) fenestrations are extremely rare, with only four cases described in the literature. The embryological mechanism leading to ICA fenestration is merely a hypothesis. Thus, some authors question its existence. The differentiation between an ICA fenestration and pseudofenestration (dissection with persistent true and false lumina) is a serious matter given the possibility of neurological deterioration with the latter and the potential need for endovascular intervention and antiplatelet therapy. We here present the interesting case of a middle-aged patient who presented with acute stroke symptoms and was found to have an intracranial hemorrhage on non-contrast head CT as well as an unusual, somewhat tortuous appearance of the distal left ICA.