Abstract: Genetic elastic fiber diseases arise from inherited or de novo mutations in genes encoding elastic fiber components, such as elastin, fibrillin-1, and associated proteins, leading to abnormalities in their deposition, structure, or degradation. Historically, research has focused on systemic, non-neurological manifestations, which are more clinically apparent and often life-threatening, particularly cardiovascular complications. In contrast, potential involvement of the central nervous system (CNS) has received limited attention, even though the brain and spinal cord are richly vascularized structures, extensively perfused, and critically dependent on the integrity of their blood vessels.