To assess the autonomic regulation features in patients with connective tissue dysplasia (CTD) 55 patients (18males and 37 females, mean 23±7.25 years old) were included into the study. A statistically significant heart rate parasympathetic influences reduction was observed in combination with a considerable sympathetic autonomic nervous system contribution in the cardiovascular activity regulation. The results confirm that initially low level of the vagal tonus in patients with CTD is unable to fully restrain the growth of sympathetic activity in orthostasis and, apparently, in all other situations, accompanied with physiological autonomic tonus activation.