The authors present a case in which a posterior communicating artery (PCoA) infundibulum progressed into an aneurysm in a patient with Alagille syndrome (arteriohepatic dysplasia). The 3-mm PCoA infundibulum had been noted on angiography studies obtained 5 years earlier, prior to clip occlusion of a basilar tip aneurysm. Recently, the patient presented to the emergency department with the sudden onset of headache and decreased mental status. A computerized tomography scan of the head with three-dimensional angiography revealed no gross subarachnoid hemorrhage, but did demonstrate a 5-mm PCoA aneurysm. Lumbar puncture demonstrated xanthochromia and a large quantity of red blood cells. The patient underwent open surgery for aneurysm clip occlusion and obtained a good recovery. This case illustrates the small but growing number of examples of infundibulum progression. It also indicates the need for a close follow up in patients with congenital abnormalities that may pose an increased risk for what has traditionally been considered a benign lesion.