This is a case report of an unusual aneurysm of the distal anterior inferior cerebellar artery. A 44-year-old woman had a severe frontal headache and vomiting of sudden onset. On the day after admission, the patient began to demonstrate nuchal rigidity and difficulty with upward gaze bilaterally. There were no complaints at this time suggestive of a syndrome of the cerebellopontine angle. A computed tomographic scan revealed an intraventricular hemorrhage of the 3rd and 4th ventricles; however, multiple attempts at four-vessel angiography were required before an aneurysm could be demonstrated. On the 28th hospital day, a suboccipital craniectomy using the retromastoid approach in the lateral position was performed. A saccular aneurysm with a surrounding hematoma in the distal anterior inferior cerebellar artery was found and clipped. The operative findings revealed that the aneurysm was unrelated to the cerebellopontine angle. After surgery, the patient had an uneventful recovery and complete resolution of symptoms.