This article discusses recent advances, ongoing challenges, and distinct approaches for caring for infants and children with pulmonary arterial hypertension (PAH). It highlights the unique features of pediatric PAH compared to adult disease, emphasizing the need for tailored diagnostic, prognostic, and therapeutic strategies. The authors provide an updated risk stratification tool and treatment algorithm, including strategies for patients with associated cardiopulmonary conditions. They address the lack of randomized controlled clinical trials in pediatric PAH, particularly in neonatal and infant populations, and discuss the management of children failing targeted PAH therapy, including balloon atrial septostomy, lung transplantation, and pulmonary-to-systemic shunt (Potts). The article also introduces a novel multimodal approach for managing PAH associated with congenital heart diseases (CHD) with borderline pulmonary vascular resistance. Additionally, it emphasizes the importance of a complete diagnostic workup and the role of echocardiography in neonatal PH. The authors propose a pragmatic treatment algorithm based on risk-oriented approaches and highlight the need for interdisciplinary care and individualized treatment plans. They also discuss the challenges in drug approval and the importance of genetic testing and counseling in pediatric PAH.This article discusses recent advances, ongoing challenges, and distinct approaches for caring for infants and children with pulmonary arterial hypertension (PAH). It highlights the unique features of pediatric PAH compared to adult disease, emphasizing the need for tailored diagnostic, prognostic, and therapeutic strategies. The authors provide an updated risk stratification tool and treatment algorithm, including strategies for patients with associated cardiopulmonary conditions. They address the lack of randomized controlled clinical trials in pediatric PAH, particularly in neonatal and infant populations, and discuss the management of children failing targeted PAH therapy, including balloon atrial septostomy, lung transplantation, and pulmonary-to-systemic shunt (Potts). The article also introduces a novel multimodal approach for managing PAH associated with congenital heart diseases (CHD) with borderline pulmonary vascular resistance. Additionally, it emphasizes the importance of a complete diagnostic workup and the role of echocardiography in neonatal PH. The authors propose a pragmatic treatment algorithm based on risk-oriented approaches and highlight the need for interdisciplinary care and individualized treatment plans. They also discuss the challenges in drug approval and the importance of genetic testing and counseling in pediatric PAH.