Objectives: To provide an updated synthesis of the evidence on the management of pulmonary hypertension (PH) in pediatric patients. Methods: We performed a thorough search of electronic databases such as PubMed, Science Direct, Cochrane Library, and Scopus. Two independent reviewers evaluated and retrieved information from qualifying papers. Results: Our data consists of eight studies with 2003 children, 930 (46.3%) of whom were female. Prostacyclin agonists were found to significantly improve hemodynamic parameters and reduced BNP levels, which implies that they have promising clinical efficacy and a good tolerance profile. No major side effects were noted except for headaches and gastrointestinal symptoms. Systemic glucocorticoids, inhaled NO, endothelin receptor antagonists, and PDE showed tolerability and clinical improvements in pediatric PH. Side effects such as acute kidney damage, renal failure, and methemoglobinemia were noted with the inhaled NO. Conclusion: Pediatric PH is still a difficult condition with high morbidity and mortality. All of the reported pharmacological interventions demonstrated good clinical outcomes and almost no adverse effects. Healthcare practitioners can improve the care of children with PH and eventually improve their quality of life and long-term survival by incorporating new research into clinical practice.