A persistent hypoglossal artery was first described in 1889 and is one of the more common anatomical variations arising from aberration in normal development. Endovascular coiling has been recognised as a robust treatment for acutely ruptured intracranial arterial aneurysms, although specific data regarding an aneurysm arising from a persistent hypoglossal artery is lacking due to the low incidence. Here we report both the oldest patient reported to be treated with a persistent hypoglossal artery-associated aneurysm and also explicitly report endovascular treatment of a persistent hypoglossal artery aneurysm arising at the posterior inferior cerebellar artery origin. Qualitative systematic review of the available medical literature demonstrates limited evidence regarding treatment of persistent hypoglossal artery-associated aneurysms with the majority being carried out via open surgery. Ruptured posterior inferior cerebellar artery aneurysm arising from a persistent hypoglossal artery can be successfully and safely treated by endovascular therapy via the persistent hypoglossal artery. Randomised study of this situation is unlikely to be feasible; however, qualitative review of the literature reveals six such aneurysms that have been treated surgically, and this case appears to be the first via an endovascular means.