We report a rare case of a ruptured dissecting anterior inferior cerebellar artery (AICA) aneurysm treated by endosaccular embolization with a Guglielmi detachable coil (GDC). An 85-year-old female presented with headache. Computed tomographic (CT) scan showed subarachnoid hemorrhage and intraventricular hemorrhage in the fourth ventricule. Cerebral angiography and 3D-CT angiography revealed an aneurysmal dilatation at the anterior pontine segment of the right AICA with a diagnosis of arterial dissection. The right posterior inferior cerebellar artery (PICA) was absent and the right AICA supplied the territory normally nourished by the right PICA. The aneurismal dilatation was occluded by endosacullar embolization with preservation of the AICA. The distal AICA aneurysm is rare and only seven cases treated with endovascular embolization have been reported. In these, six cases were treated by parent artery occlusion with coil and the subsequent three cases presented with ischemic complications. Only one case was treated by endosaccular embolization with GDC. To our knowledge, this is the second report of the distal AICA aneurysm treated by endosaccular embolization with GDC. Distal AICA aneurysms are briefly discussed while reviewing the literature.