To evaluate the role of tissue Doppler echocardiogram and insulin like growth factor binding protein type 2 in diagnosis and evaluation of severity of pediatric pulmonary hypertension. Methods: This cross-sectional case, control study included 80 children divide into two equal groups: Group (A): patients with PHT diagnosed by Echocardiogram as mean pulmonary artery pressure more than 25 mmHg (MPAP>25). Group (B): age and sex matched to healthy patients . Detailed clinical assessments, tissue Doppler echocardiographic examinations, and IGFBP-2 measurements using ELISA were conducted. Results: There is RV dilataion and disfunction ( higher MPI ) more in cases group . ROC analysis revealed a significant excellent AUC of 0.923 for IGFBP2 with a 95% CI = 0.862 – 0.984 (P < 0.001). The best cutoff point was >168 mg\L, at which sensitivity and specificity were 95% and 77.5%, respectively for prediction of PH. IGFBP2 showed significant positive correlations with MPI of RV (r = 0.619, P < 0.001). Conclusion: PHT was associated with RV dilatation and dysfunction wither systolic, and diastolic cmfirmed by high MPI of RV. IGFBP2 is an excellent predictor of PH which sensitivity and specificity were 95% and 77.5%, respectively IGFBP2 is positively associated with severity of PH showed significant positive correlations with conventional echocardiographic and tissue doppler parameters