A 42-year-old woman experienced the sudden onset of a severe headache. Angiograms demonstrated a persistent primitive hypoglossal artery (PHA) originating from the internal carotid artery at the C-2 vertebral level. In addition, a fenestration at the PHA-basilar artery (BA) junction and an aneurysm at the proximal end of this fenestration were revealed. To perform endovascular embolization of the aneurysm, a microcatheter was introduced into the aneurysm sac via the PHA and two Guglielmi Detachable Coils were placed in the aneurysm. The patient's postoperative course was uneventful, and she was able to resume her normal life. Although many clinical cases have been reported in which a ruptured aneurysm was associated with a PHA or a BA fenestration, as far as the authors know there has been no case in the literature in which a ruptured aneurysm associated with both anomalies and no case in which endovascular embolization was used to treat a ruptured aneurysm associated with a PHA. This rare case is discussed and a review of the relevant literature is presented.