There is increasing amount of evidence pointing toward a high prevalence of psychiatric conditions among individuals with hypermobile type of Ehlers-Danlos syndrome (JHS/hEDS). A literature review confirms a strong association between anxiety disorders and JHSh/hEDS, and there is also limited but growing evidence that JHSh/ hEDS is also associated with depression, eating, and neuro-developmental disorders as well as alcohol and tobacco misuse. The underlying mechanisms behind this association include genetic risks, autonomic nervous system dysfunction, increased exteroceptive and interoceptive mechanisms and decreased proprioception. Recent neuroimaging studies have also shown an increase response in emotion processing brain areas which could explain the high affective reactivity seen in JHS/hEDS. Management of these patients should include psychiatric and psychological approaches, not only to relieve the clinical conditions but also to improve abilities to cope through proper drug treatment, psychotherapy, and psychological rehabilitation adequately coupled with modern physiotherapy. A multidimensional approach to this "neuroconnective phenotype" should be implemented to ensure proper assessment and to guide for more specific treatments. Future lines of research should further explore the full dimension of the psychopathology associated with JHS/hEDS to define the nature of the relationship. © 2017 Wiley Periodicals, Inc.KEY WORDS: joint hypermobility; anxiety; psychopathology; neuroconnective phenotype; hypermobile Ehlers-Danlos syndrome Professor Antonio Bulbena, M.D., M.Sc, Ph.D., is the Chair of the Department of Psychiatry at the Autonoma University of Barcelona with clinical, academic, and administrative contributions particularly in the area of psychosomatic medicine and anxiety disorders, dementia, chocolate and carbohydrates, clinical measurement in psychiatry, phobias, seasonality, and biometeorology. Has recently developed the Neuroconnective Phenotype and has published numerous books, book chapters, and scientific articles in peer-reviewed journals.Carolina Baeza-Velasco, Ph.D., is a clinical psychology at the Paris Descartes University, with important contributions in the area of psychological assessment and treatment of patients with comorbid anxiety disorders and joint hypermobility among other conditions. Has published several articles about the psychological factors of EDS and related conditions. Andrea Bulbena-Cabre, M.D., M.Sc., is a Psychiatry Research Fellow at the Icahn School of Medicine at Mount Sinai/J. J. Peters Bronx VA Hospital. She has specialized in psychosomatic medicine and is currently studying the anxiety-joint hypermobility phenomena in bipolar and psychotic spectrum disorders. Other research interests include substance abuse, especially in synthetic cannabis and psychosis.Guillem Pailhez, M.D., Ph.D., is an Assistant Professor at the Department of Psychiatry at the Autonoma University of Barcelona, has devoted his career in the study of the interactions between mind and body ...