Introduction: To determine cardiovascular features in children with connective tissue dysplasia. Methods: The study included 85 children, 47 girls and 38 boys, average age 13 years. Patients were evaluated by clinical and paraclinical aspects (electrocardiography, Doppler echocardiography and Holter 24 hour monitoring electrocardiography). Results: The most common clinical findings were chest pain (87.50%) and palpitations (48.50%). Fenotypical characteristic prevailed for articular hipermobility (96%), asthenic constitutions (53.70%), low development of muscular system (48.75%), with positiv Beighton criteria in 86.65% of cases. The results from ECG have established sinus tachycardia (52.50%), supraventricular extrasystoles (51.25%) and ventricular extrasystoles (27.50%). Echocardiography revealed MVP first-degree (71.25%) and false tendon in the left ventricle (53.75%). Holter ECG results showed predominance: synus tachycardia (56.25%), supraventricular extrasystoles (52.50%) mostly 50 – 100/24 h (46.25%) and ventricular (37.50%), first-degree ventricular extrasystoles (25%) and second-degree Lown ventricular extrasystoles (7.5%). Conclusions: The most common clinical findings in patients were chest pain and palpitations. The results of the research have highlighted the clinical connection between positive major Beighton criteria (86.65%) and the connective tissue dysplasia signs. Echocardiography revealed the prevalence of the MVP first-degree. The Holter ECG 24 h highlighted supraventricular and ventricular extrasystoles in children with connective tissue dysplasia.