A patient with a ruptured distal medial lenticulostriate artery (mLSA) aneurysm presenting with intraventricular hemorrhage was successfully treated using endovascular treatment. Case Presentation: A 60-year-old woman presented with impaired consciousness. Radiological examination revealed intraventricular hemorrhage caused by a rupture of a distal mLSA aneurysm. Using endovascular technique, approaching contralaterally through the anterior communicating artery (AComA), complete occlusion of the aneurysm was achieved by N-butyl-2-cyanoacrylate (NBCA) injection. The postoperative course was uneventful. Conclusion: Intraventricular aneurysms at a distal site of the perforating arteries are rare. Although there have been reports on patients with distal mLSA aneurysms treated by open surgery or conservative therapy, endovascular therapy should also be considered as a treatment option. Keywords▶ intraventricular hemorrhage, intraventricular aneurysm, medial lenticulostriate artery, endovascular therapy, N-butyl-2-cyanoacrylate This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives International License.