Disturbances in the formation of connective tissue lead to significant pathological changes in both individual organs and tissues, and at the organismal level. The complexity of diagnostics is also connected with the fact that there is no single terminology, a single view of the diagnostic criteria, a single approach among doctors of different specialties. The prevalence of external phenotypic signs of connective dysplasia is quite high, which can lead to overdiagnosis. On the other hand, insufficient attention to the manifestations of dysplasia can lead to delayed diagnosis, which can cause adverse complications. The most studied are clinical manifestations in dysplastic changes in the cardiovascular system, musculoskeletal system. This article provides an overview of current data on changes in the nervous system. Sufficient attention was paid to the pathology of the nervous system in differentiated forms (Marfan syndrome, Ehlers—Danlos, etc.). Currently, the role of various vascular anomalies, aneurysms associated with undifferentiated forms of connective tissue dysplasia is widely discussed. Much attention is also paid to clinical manifestations of the autonomic nervous system: sympathicotonic manifestations predominate in connective tissue dysplasia. There is evidence of an association of headaches, musculoskeletal pain, and connective tissue dysplasia in both children and adults.