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Introduction: A potentially fatal consequence of adult sickle cell disease (SCD) is pulmonary hypertension (PHT). However, nothing is known regarding how common pulmonary hypertension is in the pediatric SCD population. Aims: To find out the prevalence of pulmonary hypertension in children with SCD. Materials and method: The present study was an Observational Cross-sectional study. This Study was conducted from November 2020 to October 2022 at department of Pediatrics and Sickle cell institute, Veer Surendra Sai Institute of Medical Sciences and Research, Burla. Total of 552 patients were included in this study. Result: In our study, the overall prevalence of pulmonary hypertension is 20.4% (113/552). Of them, 77 (13.9%) have moderate pulmonary hypertension and 36/113 (6.5%) have mild hypertension. Pulmonary hypertension was substantially correlated with low hemoglobin (p=0.021), a high number of crises (p=0.000), a high number of blood transfusions (p=0.000), the existence of a loud second heart sound (p=0.000), and the presence of parasternal heave (p=0.000). With age, there was a tendency toward an increase in the prevalence of pulmonary hypertension in children with sickle cell disease. Conclusion: Providing fresh perspective on the prevalence of pulmonary arterial hypertension in children with sickle cell anemia in Western Odisha was the goal of this dissertation. Additionally, a comparison between our study location and the national average for the prevalence of pulmonary arterial hypertension among children with sickle cell anemia was sought. The findings of this study show that children with sickle cell anemia in the age range of 5 to 14 years had a prevalence of 20.4% pulmonary arterial hypertension. In older male sickle cell anemia children, pulmonary arterial hypertension is more prevalent. Depending on the demographic and diagnostic method, children with sickle cell anemia have varying rates of pulmonary hypertension. [ABSTRACT FROM AUTHOR]