Tuberous sclerosis is a hereditary connective tissue disorder affecting multiple organs and is associated with a wide variety of abnormalities of the central nerve system. The symptoms vary according to the organ involved and may appear in later life. In connective tissue disorders, vascular anomalies are frequent, though cerebrovascular anomalies are extremely rare. We recently, encountered a case of posterior inferior cerebellar artery aneurysm associated with tuberous sclerosis. This report describes clinical and radiological details of this case and includes a brief review of the literature.