This is a cross-sectional study done on 50 Egyptian children aged 6 years and above who followed up at the Rheumatology Clinic at Specialized Hospital - Cairo University ; they were diagnosed clinically and by laboratory tests to have connective tissue disease. Doppler echocardiography was done for all the enrolled patients to detect the prevalence of PAH in cases with CTD. A Pulmonary artery systolic pressure (PASP) 25mmHg was taken as a cut off level for diagnosis of pulmonary hypertension. Thirty one out of fifty (62%) CTD patients Group1were diagnosed as having Pulmonary hypertension while nineteen out of fifty (38%) CTD patients Group 2 not associated with increase in PASP.